Famciclovir-resistant disseminated herpes zoster

A man in his 40s was admitted to the hospital with a 10-day history of edema and presented with vesicles and bullae on his right cheek, neck, back, both eyelids, and the dorsal surface of his hands (Figure 1A)

Prior to presentation, he had been treated for herpes zoster with famciclovir for 1 week, during which the lesions kept developing and were accompanied with irregular fever (maximum temperature, 39°C)

Physical examination revealed edema on both eyelids

multiple papules, blisters, and crusted erosions with thin exudation on his right cheek and neck

and erythematous plaques and tense blisters and bullae on his back and on the dorsal surface of both hands (Figure 1B)

A full laboratory workup was performed for autoantibodies for systemic lupus erythematosus (SLE), pemphigus, and bullous pemphigoid

skin and bone-marrow biopsies and direct immunofluorescence were performed

and immunohistochemichal analysis, a swab of exudation, and bacterial, fungal, and atypical mycobacterial cultures from blood and tissue were also examined

multiple papules, blisters, and crusted erosions with thin exudation on right cheek and neck

B, Erythematous plaques, tense blisters, and bullae on both hands (right hand depicted)


Telangiectatic metastatic breast carcinoma

A woman in her 60s presented to the clinic with a 3-month history of pain, swelling, and erythema on her face

Eleven years earlier, she had been diagnosed with left breast carcinoma and had undergone a mastectomy with subsequent radiotherapy and chemotherapy

Seven years later, she had been found to have left supraclavicular lymph nodes metastasis and had undergone a surgical excision

On presentation for the current problem, the patient experienced pain on palpation, but denied fever, chills, or other symptoms

A physical examination revealed violaceous swelling with telangiectasia and necrosis on the forehead, eyelids, nose, and cheek, predominantly involving the left side of the face (Figure 1)

A biopsy of the lesion was performed for histopathological analysis (Figure 2)

A, A full image and B, a three-quarters image of the face, showing telangiectatic, swelling erythema mainly involving the left side of the forehead, eyelids, nose, and cheek


A man in his 50s presented with an abdominal skin eruption of 2 days’ duration

His medical history included hepatitis C, cirrhosis, and an orthotopic liver transplant (OLT) 3 years prior that was subsequently treated with tacrolimus, 5 mg, and mycophenolate, 750 mg, twice daily

Approximately 2½ years after the transplant, the patient developed gastric outlet obstruction secondary to an infiltrative gastric wall mass

The gastric wall mass was found to be associated with plasmablastic posttransplantation lymphoproliferative disorder (pPTLD)

He was subsequently instructed to stop use of mycophenolate and decrease use of tacrolimus to 0.25 mg daily, then treated with 1 cycle of CHOP chemotherapy (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone) and lenalidomide, resulting in symptomatic and radiologic improvement

Five weeks after treatment with CHOP chemotherapy, the patient presented with an acute-onset abdominal skin eruption without fever or pain

A computed tomographic scan of the abdomen and pelvis showed possible abdominal wall cellulitis, so treatment with piperacillin-tazobactam and vancomycin was empirically started

Physical examination revealed confluent indurated violaceous papules and plaques coalescing into an annular pattern encircling the healed OLT incisional scar with central sparing (Figure 1A)

A 4-mm punch biopsy specimen was obtained for histopathological evaluation (Figure 1B and C) and bacterial and fungal cultures

A, Confluent, indurated, violaceous papules and plaques coalescing into an annular pattern encircling the well-healed orthotopic liver transplant incisional scar

B, Hematoxylin-eosin staining of the punch biopsy shows an atypical, hyperchromatic infiltrate present throughout the superficial and deep dermis

C, High magnification reveals crushed but markedly atypical-appearing lymphoid cells with nuclear hyperchromasia and scant amounts of cytoplasm

Multiple mitotic and apoptotic figures are easily identified


Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes

A 13-year-old boy with a longstanding history of gait imbalance presented with 2 episodes of acute-onset left hemibody weakness and dysarthria without changes in sensorium in a 24-hour period

Symptoms lasted approximately 60 minutes each before completely resolving

prior to the day of the events, he had never experienced similar phenomena

Initial examination following the second event revealed mild-to-moderate dysarthria, pes planovalgus and tight achilles tendons bilaterally, and decreased vibratory and fine touch sensation from the great toe up to the mid shin bilaterally

Reflexes were absent at the patella and achilles bilaterally

Bilateral dorsiflexion weakness was present (4 of 5)

There were otherwise no motor deficits, muscle atrophy, or lateralizing neurologic abnormalities

On arrival, the patient underwent a stroke/transient ischemic attack workup

Results of a comprehensive metabolic panel, complete blood cell count, erythrocyte sedimentation rate, C-reactive protein, and a coagulation panel were within normal limits

Computed tomography angiogram demonstrated no evidence of extra or intracranial dissection

Magnetic resonance imaging of the brain with and without contrast (Figure) demonstrated symmetric, bilateral, restricted diffusion, with T2 hyperintensities in the supratentorial white matter and corpus callosum

Lumbar puncture was obtained and revealed normal cell counts and no oligoclonal bands or evidence of infection

A, Diffusion-weighted imaging sequence demonstrating bilateral, symmetric, restricted diffusion of the supratentorial white matter (corresponding hypointensity noted on apparent diffusion coefficient sequences)

B, T2 sequence demonstrating bilateral and symmetric increased signal in the supratenorial white matter–sparing U-fibers

The patient had reportedly always walked on the outside of his feet and had been considered clumsy when compared with other children but had no history of motor delays

Family history revealed a mother with diminished reflexes, hand tremor, and balance issues with a prior inconclusive electromyogram  and nerve conduction study

A younger brother with attention-deficit/hyperactivity disorder also had similar gait abnormalities


A white man in his 20s presented with numerous pink and brown papules and plaques, some with ulceration, on the head and along the midline of the neck and spine (Figure)

Medical history included medulloblastoma treated with irradiation of the brain and spine at age 2 years

He also received radiation therapy for a meningioma of the posterior fossa in adulthood

He was also treated for several seemingly similar lesions on the face and scalp and had a family history of similar cutaneous lesions reported in 2 brothers and 1 niece

Figures show crusted erythematous papules and plaques adjacent to the surgical excision scar within a prior irradiation field on the head and neck (A) and erythematous and hyperpigmented papules and plaques overlying diffuse postsurgical scarring within a prior irradiation field on the back (B)


A 41-year-old woman with a history of Hodgkin disease in her adolescence was diagnosed with melanoma of the left upper thigh that was classified as American Joint Committee on Cancer (AJCC) stage IIA with (pT3aN0 [sn0/1] M0) BRAF mutation

One year later she underwent inguinal lymphadenectomy for macrometastasis followed by adjuvant immunotherapy with ipilimumab, a monoclonal antibody that targets cytotoxic T-lymphocyte antigen 4. Owing to local disease progression, treatment escalation with combined immunotherapy (ipilimumab and nivolumab, an anti–PD-1 [programmed cell death 1]) antibody was initiated

Apart from immunotherapy-induced thyroiditis, the treatment was well tolerated

However, after 11 cycles of nivolumab, the patient presented with progressive painful reddening and swelling of the left thigh

There was no deterioration of the general condition, no fever, no change in current medication, and no recent cold exposure

Clinical examination revealed diffuse erythematous induration of the upper thigh expanding to the lower left abdominal area, as well as multiple small palpable subcutaneous nonindurated nodules on the ipsilateral gluteal region and leg (Figure, A)

Fluorine 18 (18F) fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET-CT) detected multiple FDG-active lesions in both lower extremities and the lumbar region

a previously described left iliac lymphadenopathy was partially regressed (Figure, B)

A diagnostic biopsy specimen of the left thigh revealed a lobular lymphocytic panniculitis with dense infiltrate of mainly lymphocytes and plasma cells with no signs of vasculitis (Figure, C and D)

Direct immunofluorescence and blood analysis showed an absence of specific autoimmune antigens (test results for α-1 antitrypsin, antinuclear antibody, anti-Sjögren syndrome–related antigen, and anti-Sjögren syndrome–related antigen B were all unremarkable)

A, Localized erythematous brownish induration seen on the upper thigh

Arrowheads correlate with the most prominent clinical findings

B, Radioactive fluorine 18 (18F) fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET-CT) reveals multiple subcutaneous lesions in both lower extremities, the lumbar region, and on the ipsilateral lower leg with high FDG uptake highly suggestive of (in transit) metastasis

C and D, Lesional skin biopsy samples obtained from the proximal left thigh show normal epidermis and dermis with patchy infiltrate of the deep subcutaneous fat (hematoxylin-eosin stain)

Green arrows indicate the plasma cells, and blue arrows indicate the lymphocytes


A white man in his 70s presented with a gradually enlarging right supraorbital mass that had been neglected for 4 years

His comorbidities included congestive heart failure, atrial fibrillation, coronary artery disease, chronic obstructive pulmonary disease, hypertension, and a 50-pack-year history of tobacco abuse

Physical examination revealed a firm, nonmobile, nontender, bulging mass situated over the right frontal sinus, which expanded to the upper right eyelid

The mass covered the globe completely, resulting in total vision obstruction (Figure, A)

However, the patient’s vision remained intact when the mass was elevated off the eye with great effort

His right pupil was round and reactive to light without afferent pupillary defect

A non–contrast-enhanced computed tomography (CT) scan demonstrated a large, right supraorbital mass with bony destruction of the anterior table of the right frontal sinus (Figure, B)

It extended into the superior aspect of the orbit, displacing the right globe inferiorly

Air was seen within the mass (Figure, C)

A fine-needle aspiration biopsy yielded inconclusive results

After medical clearance, the patient was taken to the operating room for a wedge excisional biopsy with decompression of the right supraorbital mass under monitored anesthesia care

Histopathological investigation revealed large nests of neoplastic epithelioid cells surrounded by fibrotic tissue and necroinflammatory debris

The neoplastic cells contained round nuclei with single prominent nucleoli and abundant clear to vacuolated cytoplasm (Figure, D)

B, Computed tomography (CT) scan of destruction of the anterior table of right frontal sinus

D, Neoplastic epithelioid cells of the mass (original magnification ×40)


A 46-year-old African swimmer presented with a 1-month history of progressive left hearing loss and vertigo

He also reported asthenia, sleep disorders, and urinary frequency

The urinary frequency has been treated for 2 years with many drugs (tamsulosin, desmopressin, and solifenacin) without improvement

The patient had spent his childhood in eastern Africa, where he swam in Lake Tanganyika

His history did not reveal trauma, infection, or any other associated conditions at the onset of the complaints

The ear, nose, and throat examination revealed a right horizontal nystagmus and a poorly delineated hypoesthesia of the left pinna

He could not walk properly owing to his vertigo

The rest of the clinical examination findings were unremarkable

Additional examinations (ie, pure-tone audiometry, video nystagmography, tympanometry) revealed left profound hearing loss with an air-bone gap of 40 dB and central vertigo

Otoacoustic emissions were absent on the left side

Auditory brainstem responses showed asymmetric interpeak latencies of waves I through V and III through V, suggesting slower conduction on the left side

Computed tomography and cerebral magnetic resonance imaging (MRI) revealed a 24 × 20-mm hemorrhagic lesion in the left middle cerebellar peduncle associated with a thickening of the cochleovestibular nerve (Figure)

The patient was referred to urology, where a 13 × 8-mm mass was discovered in the lateral wall of the prostate gland

Biopsies of prostate and brain lesions, blood tests, and urinalysis were performed

Computed tomography (CT) scan and magnetic resonance images (MRIs) of the left middle cerebellopontine angle and prostate gland

A, Axial CT scan showed a hyperdense lesion suggesting hemorrhage in the left middle cerebellar peduncle

B, The lesion extended to the base of the vestibulocochlear nerve that was thickened (arrowheads) on the Fast Imaging Employing Steady-State Acquisition (FIESTA) MRI (axial plane)

C, Coronal postcontrast MRI demonstrated a T1 hyperintense lesion that was consistent with hemorrhagic lesion with no true enhancement

D, Axial pelvic MRI showed a prostatic lesion (13 × 4 mm) located in the left posterior and lateral wall (arrowhead)

the prostatic volume and size were substantially increased (132 mL, 59 × 60 × 70 mm)


A preschool boy presented with asymptomatic, reddish, raised lesions over his right ear, noticed 3 months earlier, associated with multiple painless swellings on the ipsilateral side of the neck for the past 3 weeks

On examination, there were multiple, erythematous papules coalescing to form a plaque with areas of scarring over the posterior aspect of right ear, along with firm, nontender posterior cervical lymphadenopathy (Figure, A)

There was no history of trauma or intervention prior to the onset of lesions

His medical history was unremarkable, as were the rest of the mucocutaneous and systemic examination findings

A skin biopsy from the plaque was submitted for histopathological examination (Figure, C and D)

A, Erythematous papules coalescing to form a plaque with areas of scarring, along with ipsilateral posterior cervical lymphadenopathy

B, The lesions have healed with atrophic scarring after 6 weeks of treatment

C, Microphotograph showing unremarkable epidermis with evidence of granulomatous inflammation and central caseous necrosis in the dermis (original magnification ×100)

D, Ziehl-Neelsen stain shows acid-fast bacilli (oil immersion


A man in his 60s presented with an approximately 4-month medical history of lip enlargement with nodular growths

Skin examination was significant for multiple erythematous and flesh-colored papules and nodules symmetrically affecting the upper and lower lips with extension onto the vermilion border (Figure 1A)

There were no intraoral lesions, and all other findings of his examination were within normal limits

The patient denied fevers, chills, weight loss, night sweats, cough, lymphadenopathy, abdominal pain, change in bowel habits, or other systemic symptoms

A biopsy was obtained of the vermilion lip (Figure 1B)

A, Symmetric lip enlargement with infiltrative erythematous and skin-colored papules and nodules extending onto the vermilion border

B, Hematoxylin-eosin stain (original magnification ×40)


A woman in her 50s with a right lower-eyelid yellow plaque presented with unremitting abdominal and back pain

Computed tomographic (CT) scan of the chest, abdomen, and pelvis demonstrated extensive infiltrative stranding from the thorax through the retroperitoneal space

Sclerotic changes in multiple osseous structures were noted

Idiopathic retroperitoneal fibrosis was diagnosed following biopsies

Prednisone and azathioprine were started

Biopsy of the lower-eyelid yellow plaque returned xanthelasma (Figure, A)

Ureteral obstruction, chronic kidney disease, and lymphedema complicated her course

Prednisone was slowly tapered but she relapsed 3 years later

A second retroperitoneal biopsy exhibited fibroadipose tissue, foamy macrophages, and chronic inflammation with fibrosis

Azathioprine and prednisone were restarted

She required multiple surgeries for spinal stenosis and ureteral obstruction

Then, owing to knee pain, a plain radiograph was obtained which demonstrated osteosclerosis of the distal femur

Consequently, the previous eyelid and retroperitoneal biopsies were reviewed

The clinicopathologic presentation was confirmed with a molecular test

A, Clinical image of a soft, nonscaly yellow-tan plaque on the medial canthus

B, Hematoxylin-eosin stain image demonstrating foamy histiocytes with Touton giant cells (original magnification ×100)

C, CD68 immunohistochemical stain diffusely positive in the histiocytic infiltrate (original magnification ×100)

D, Computed tomographic image with contrast demonstrating retroperitoneal stranding and fibrosis with hydronephrosis (arrowheads indicate hairy kidney appearance of fibrosis)


A Japanese man in his 50s presented with a 6-month history of worsening pruritic hand dermatitis that progressed to involve his face, trunk, upper extremities, and feet

He had emigrated from Japan more than 10 years earlier but had no history of recent travel

He took no medications, had no significant medical or surgical history, and denied personal and family history of atopy

While he worked as a biomedical researcher, he denied any caustic exposures

On physical examination, his face, trunk, upper extremities, palms, and soles were marked by 1- to 2-mm erythematous papules and pustules coalescing into large, partially indurated and polycyclic plaques with centrifugal extension and occasional central clearing (Figure, A and B)

Treatment with potent topical corticosteroids was ineffective

Punch biopsies of lesions on the shoulder (Figure, C) and foot (Figure, D) were performed

A, Annular erythematous plaque studded with papules and pustules on the cheek and temple

B, Erythematous plaques studded with papules and pustules on the shoulder

C and D, Lesional specimens under hematoxylin-eosin stain taken from the shoulder (C, original magnification ×400) and plantar foot (D, original magnification ×40)


Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

A 42-year-old white woman with a history of episodic migraine with visual aura presented for evaluation of transient right upper extremity weakness and word-finding difficulty associated with headache

She admitted to having developed new intermittent vertigo and more frequent and severe throbbing migraine with transient blurry vision and nonpulsatile tinnitus several months prior

She had recently developed hearing loss with a robotic quality of auditory perception

A neurologic examination revealed diminished hearing to a finger rub on the right side

Fundoscopy showed several segmental retinal arterial plaques, with the right side worse than the left

A brain magnetic resonance image (MRI) with gadolinium revealed multiple white-matter lesions that were hyperintense on diffusion-weighted imaging (DWI), including some with enhancement, as well as a small DWI-negative lesion in the left thalamus and corpus callosum that was hyperintense on T2/fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR

An MRI of the spine with gadolinium did not reveal a signal abnormality in the spinal cord

Infectious, rheumatologic, and hypercoaguable test results were negative

A lumbar puncture revealed a white blood cell count of 8 cells/μL (normal range, 0-5 cells/μL

to convert to cells × 109/L, multiply by 0.001) with 78% lymphocytes and 22% monocytes (to calculate these as proportions of 1.0, multiply by 0.01), and a protein level of 0.067 g/dL (normal range, 0.015-0.045 g/dL

to convert to grams per liter, multiply by 10)

Cerebrospinal fluid (CSF) glucose and IgG index test results were normal, with culture and oligoclonal bands negative

An audiogram revealed bilateral sensorineural hearing loss, with the right side worse than the left

Retinal fluorescein angiography was obtained

A, Pink arrowheads indicate hyperintensities in the right centrum semiovale

B, The yellow arrowhead indicates a hyperintensity in the corpus callosum


Advanced cutaneous T-cell lymphoma/Sézary syndrome

A white woman in her 70s with advanced Alzheimer disease was referred to the hematology clinic for evaluation of a high hemoglobin level (169 g/L

normal range, 120-160 g/L) and red blood cell count (5.67 × 1012/L

normal range, 3.8-4.8 × 1012/L) as well as a generalized itch that was worse after a bath

On examination, she had a florid, erythematous macular eruption over the trunk and limbs (Figure, A) but no hepatosplenomegaly or lymphadenopathy

In addition to the high hemoglobin level, mild lymphocytosis (absolute lymphocyte count, 6.2 × 109/L, range 1.5-4.0 × 109/L) was noted with the lymphocyte morphology, suggesting reactive changes

Skin biopsy specimen (Figure, B) showed a normal epidermis with a pericapillary infiltrate of small lymphocytes restricted to the dermis and no leukocytoclastic vasculitis, fungal organisms, or dermal mucin

The absence of cellular atypia and epidermal involvement suggested a diagnosis of lupus, or gyrate or annular erythema, and the need for clinicopathological correlation

B, Skin biopsy specimen shows a pericapillary infiltrate of small lymphoid cells restricted to the dermis (hematoxylin-eosin, original magnification ×200)

C, Blood smear shows a typical lymphocyte (May-Grünwald Giemsa stain, original magnification ×400)

Treatment began with a topical emollient, steroid creams, and oral antihistamines

Skin biopsy was repeated 2 months after the original procedure owing to a suboptimal clinical response, but the histological appearances were unchanged

The hemoglobin level remained high, and the lymphocyte count had increased to 9.0 × 109/L (Figure, C)


A 53-year-old African American man with a history of bipolar disorder presented to the emergency department with an enlarging right-sided tongue mass that had caused mild discomfort and halitosis over the course of 1 week

He denied recent injury, fever, chills, odynophagia, or difficulty breathing

Laboratory examination was significant for leukocytosis (white blood cell count, 15 700/μL) and hypercalcemia (total calcium level, 13.1 mg/dL) (to convert white blood cells to ×109 per liter, multiply by 0.001

to convert calcium to millimoles per liter, multiply by 0.25)

Physical examination was notable for a 4- to 5-cm pedunculated, ulcerative mass on the dorsal oral aspect of the tongue near midline, anterior to the foramen cecum, without palpable cervical lymphadenopathy

Contrast-enhanced computed tomography (CT) demonstrated a rim-enhancing, centrally hypodense lesion in the right dorsal tongue abutting the lingual septum with a somewhat ill-defined border (Figure 1)

there was no associated osseous erosion of the mandible or hard palate, no tongue prolapse to suggest hypoglossal nerve palsy, no involvement of the soft palate, and no cervical lymphadenopathy by radiological size criteria

An excisional biopsy of the anterior aspect of the mass was performed at bedside, and the patient was discharged home with instructions to follow up in the otolaryngology clinic

A, Sagittal contrast-enhanced computed tomography (CT) demonstrates an exophytic mass on the dorsal aspect of the tongue

B, Axial contrast-enhanced CT demonstrates a peripherally enhancing mass of the oral tongue, to the right of midline


A 17-year-old girl with a history of chronic sinusitis presented with left-sided epiphora, purulent drainage, and pain and right-sided jaw pain

She had previously been treated with antibiotics and mupirocin nasal irrigation, with temporary improvement

The patient and her identical twin sister had required multiple surgical procedures in the past for osseous lesions in the upper and lower jaws, including an aggressive resection via bilateral Caldwell-Luc approach 2 years previously

a computed tomographic scan from that initial presentation is shown in the Figure, A

At the current presentation, examination using flexible nasal endoscopy revealed bulging of the left lateral nasal wall

imaging revealed a 14-mm, dense, expansile, sclerotic mass with moderate calcifications obstructing the left nasolacrimal duct

a similar 2.3 × 1.8–cm lesion of the right mandibular body

and evidence of previous sinus surgery (Figure, B)

Because of these findings, left-sided endoscopic dacryocystorhinostomy and sinus surgery was performed

Histologic findings are shown in the Figure, C and D

A, Contrast-enhanced computed tomographic image at initial presentation

B, Non–contrast-enhanced computed tomographic image at 2 years after bilateral Caldwell-Luc resection showing mass in left nasolacrimal duct

C, Photomicrograph depicting a proliferation of highly cellular, fibrous connective tissue (arrow) and cementum-like hard tissue (arrowhead) (hematoxylin-eosin, original magnification ×100)

D, Photomicrograph showing whorls of spindle cells (arrow) with relatively acellular calcified masses of cemento-osseous material (arrowhead) (hematoxylin-eosin, original magnification ×200)


A woman in her 60s reported right upper lip submucosal masses that had been present for several weeks

She denied associated pain, bleeding, or recent trauma to the lip

She also denied any other constitutional symptoms

Her social history was significant for a 45–pack-year smoking history and social drinking

On physical examination, the patient had right upper lip submucosal masses without any tenderness or mucosal abnormality

The remainder of the otolaryngic examination was unremarkable

A computed tomographic scan of the neck with contrast demonstrated multiple hyperenhancing nodules

the largest was 3 × 7 × 6 mm, with no local bony erosion or cervical lymphadenopathy (Figure 1)

Computed tomographic (CT) scans of hyperenhancing nodules of the upper lip (red arrows)


A woman in her 20s presented with almost equally sized, round erythematous plaques with central pallor and confluent blisters at the periphery on both forearms

According to the patient, the lesions recurrently appeared during the last 9 years, with a symptom-free period of 2 years (Figure, A)

She could not identify or recall a specific cause for these skin lesions and denied self-inflicting them

She had been treated with antibiotics (cefuroxime) as well as with topical steroids (dexamethasone) but denied any lasting effect

Her family history was without abnormalities

She reported being vaccinated against varicella zoster virus and was not immunocompromised

A, A clinical photograph shows an annular erythematous plaque with crusts, bullae, and central pallor

B, Histopathologic specimen shows normal stratum corneum, confluent necrosis of the upper epidermal layers, some multinucleated keratinocytes in the adjacent epidermis, and papillary edema with a subepidermal blister

A moderately dense perivascular lymphocytic infiltrate with eosinophilic and neutrophilic granulocytes is present in the upper dermis (hematoxylin-eosin, original magnification x200)

A skin biopsy of the forearm (Figure, B), direct immunofluorescence, and blood samples were obtained


Confluent and reticulated papillomatosis

An adolescent girl with polycystic ovarian syndrome (PCOS) presented for evaluation of hyperpigmented skin lesions affecting her neck, upper limbs, and trunk

The lesions had started about 6 months previously as recurrent crops of pruritic erythematous papules that subsided leaving behind brownish-bluish black hyperpigmentation after each episode

On examination, there were multiple, brown, hyperpigmented patches symmetrically affecting her neck, axillae, antecubital fossa, and intermammary and inframammary areas (Figure, A)

The patches showed a reticulate pattern at the peripheries

Intermammary patches had central erythema and a few subsiding papules

Additionally, there were dark brown to bluish-gray patches with reticulation on the lower back and dorsum of the neck (Figure, B)

These patches also had a few resolving erythematous papules in the periphery

There was no history suggestive of allergic rhinitis or bronchial asthma

Her IgE levels and thyroid status were normal

Her fasting blood glucose and basal insulin levels were not raised

There was no family history of similar lesions

The rest of the skin, nails, and mucosae were all normal

Potassium hydroxide mount analysis from skin scrapings did not reveal fungal hyphae or spores

A skin biopsy was performed from lesions on the lower back, and the specimen was submitted for histopathologic analysis (Figure, C and D)

A, Illustrated are multiple brown hyperpigmented patches symmetrically affecting the neck, axillae, antecubital fossa, and intermammary and inframammary areas

The patches show a reticulate pattern at the peripheries

The intermammary patches have central erythema and a few overlying edematous papules

B, Bluish-gray pigmented patches with prominent reticulation on lower back

C, Histopathologic lesional specimen shows mild epidermal hyperkeratosis, acanthosis, multiple foci of spongiosis, and an enhanced basal layer pigmentation

There is scant lichenoid and moderate perivascular lymphomononuclear and polymorphonuclear infiltrate, basal cell vacuolization, and melanin incontinence (hematoxylin-eosin)

D, Histopathologic lesional specimen at higher power reveals spongiosis, occasional neutrophilic exocytosis (yellow arrowheads), basal cell vacuolar degeneration, and necrotic keratinocytes (black arrowheads) (hematoxylin-eosin)


Systemic-onset juvenile idiopathic arthritis

Cystic fibrosis-associated episodic arthritis

An elementary school-aged girl with cystic fibrosis (CF) presented with a 2-year history of a relapsing-remitting, evanescent, salmon-pink maculopapular cutaneous eruption associated with joint pain

Each episode of joint pain and cutaneous eruption lasted 24 to 48 hours with episodes occurring anywhere from every other day to once weekly

The patient’s mother was unable to identify any possible triggers because the episodes occurred independent of pulmonary CF exacerbations or antibiotic use

The patient’s joint pain and cutaneous symptoms resolved completely between each episode and were not associated with fever

Review of photos taken during the patient’s flares revealed a variable eruption consisting of oval-shaped, salmon-pink macules on the trunk, urticarial-appearing papules on the dorsal hands, and flagellate arrays of petechial macules on the upper medial arms (Figure 1)

This eruption was intermittently pruritic but otherwise was asymptomatic

Her joint pain recurred with each episode of cutaneous eruption and primarily affected the wrist, knee, and ankle joints bilaterally

She had been treated with naproxen during these flares, which improved her joint pain but not her cutaneous symptoms

A complete blood cell count and comprehensive metabolic panel as well as C-reactive protein and erythrocyte sedimentation rate obtained during a flare were normal

Titers for parvovirus, Epstein–Barr virus, and antistreptolysin O were also negative

A, Approximately 1-cm, oval-shaped, salmon-pink macules on the right lower abdomen and right lateral thigh

B, 3- to 4-mm pink urticarial-appearing papules on the dorsal hand

C, Flagellate arrays of nonblanching 1- to 2-mm petechial macules on the upper medial arm


A man in his late 60s with a diagnosis of type 2 diabetes for more than 10 years that was complicated by neuropathy and retinopathy presented with persistent chorea in the left upper extremity for about 1 month despite aggressive correction with insulin

He was alert and oriented with healthy vital signs

No evidence of cognitive impairments was present on bedside testing results

The patient denied any recent infections and risk for human immunodefiency virus (HIV) infection

Neurological examination results were significant for choreiform and hemiballistic movements involving the left upper extremity

His home medications were carefully reviewed to rule out any medications that could cause chorea

A review of his previous records indicated an admission to an outside facility 3 weeks beforehand with severe hyperglycemia and a hemoglobin A1c level of 14.1% (to convert to the proportion of total hemoglobin, multiply by 0.01)

Despite an aggressive correction of blood glucose levels and metabolic correction, his choreiform movements had remained unresolved

On admission, a complete electrolyte and toxicology panel, including calcium, magnesium, and phosphorus, yielded normal results

The patient’s serum glucose level was 159 mg/dL (to convert to millimoles per liter, multiply by 0.0555) and his hemoglobin A1c was 10.9% without ketoacidosis

Test results for antinuclear antibodies, lupus anticoagulants, and antiphospholipid antibodies were negative

Serum thyrotropin levels, liver function test results, and parathyroid hormone levels were healthy

Copper studies, a peripheral smear, and a routine electroencephalogram yielded normal results

A general screening for cancers yielded negative results

The patient underwent brain magnetic resonance imaging (MRI) (Figure), which showed a T1 hyperintensity in the right caudate head and a lentiform nucleus with no restricted diffusion

Oral administration of olanzapine, 2.5 mg daily, in divided doses with careful monitoring of blood glucose levels resulted in the complete resolution of chorea

Magnetic resonance imaging shows T1 hyperintensity in the right caudate head (A) and a lentiform nucleus with no restricted diffusion (B)


Impingement of the left sympathetic trunk

Transection of the left sympathetic trunk

A middle-aged man with a 25-pack-year history of cigarette smoking was in his usual state of health when he developed excessive coughing with hemoptysis, which spontaneously resolved

Two months later, he presented to a hospital with complaint of shortness of breath, cough, and chest pain

A computed tomographic image of the chest (Figure 1) identified a large, 12-cm left upper lobe mass abutting the anterior chest wall pleura and extending into the mediastinum, with mediastinal lymphadenopathy as well as several liver lesions

A biopsy revealed poorly differentiated carcinoma, consistent with a primary tumor in the lung

On presentation to our multidisciplinary clinic, he reported constant, bothersome sweating of the left forehead

On physical examination, a well-demarcated area of erythematous, perspiring skin on the left forehead was noted

A, Coronal computed tomographic image of the thorax with intravenous contrast at initial presentation, demonstrating a left upper lobe mass

asterisk depicts possible area of sympathetic nerve compression or invasion

B, Radiation planning computed tomographic image taken 2 weeks after initial presentation, demonstrating growth of the left upper lobe tumor

the orange contour, the area of sympathetic trunk compression


A previously healthy 18-year-old woman presented with a growth in the right oropharynx of 4 months’ duration

At the time of presentation, it had begun to cause changes in her voice, globus sensation, and dysphagia

The patient reported a 20-pound weight loss over the prior 3 months and decreased appetite

She denied any associated difficulty breathing, pain, fever, chills, rash, or night sweats

Despite a muffled voice, she was not in respiratory distress

Fiberoptic laryngoscopy revealed a large, partially obstructing mass in the right oropharynx protruding from the right tonsil

It descended inferiorly and was partially compressing the epiglottis

The true and false vocal cords, although partially obstructed, appeared normal and mobile

The lesion was without ulceration, exudate, or erythema

The contralateral tonsil was small and normal in appearance

The remainder of her physical examination findings were unremarkable, including no neck masses

The patient agreed to surgical excision of the mass, including tonsillectomy

Her airway was secured via an awake transoral fiberoptic intubation without difficulty

The planned procedure occurred without difficulty or complication

The specimen excised was a 6 × 3 × 2-cm mass continuous with the right tonsil (Figure 1), without involvement of any surrounding structures

A, Intraoperative photograph of a large, irregularly shaped lesion protruding from the patient’s right tonsil

B, The excised specimen measured 6 × 3 × 2 cm and was continuous with the right tonsil


Idiopathic sudden sensorineural hearing loss

A healthy man in his mid 30s residing in the northeastern United States was seen by his primary care physician in mid-June with a 2-week history of flulike symptoms

He was empirically started on doxycycline because he reported frequenting wooded areas

One week later, he developed a complete right facial nerve paralysis

He was started on a course of prednisone, with subsequent full recovery of facial nerve function

About 2 weeks later, he reported new-onset right-sided hearing loss, tinnitus, and intermittent otalgia

He was evaluated in the otology clinic approximately 3 weeks after the onset of these otologic symptoms

He had no prior otologic history and no history of noise exposure

His otologic and head and neck examination findings were unremarkable

Comprehensive audiometry was performed, which showed a mild sensorineural hearing loss from 3000 to 8000 Hz in the right ear and a mild sensorineural hearing loss at 8000 Hz only in the left ear

Given the asymmetry in the right ear, magnetic resonance imaging (MRI) of the brain and internal auditory canals (IACs) with contrast was obtained

There was abnormal linear and smooth enhancement of the right facial nerve in the superior distal IAC, labyrinthine segment, and geniculate ganglion

Discrete enhancement was also seen within the inferior aspect of the right IAC in the region of the vestibulocochlear nerve (Figure 1)

T1-weighted postcontrast axial (A) and coronal (B) magnetic resonance imaging of the brain and internal auditory canals

A, Increased enhancement of the labyrinthine segment of the facial nerve (yellow arrowhead) and geniculate ganglion (pink arrowhead)

B, Discrete linear and smooth areas of enhancement in the superior distal portion of the right internal auditory canal and the inferior aspect of the right internal auditory canal, corresponding to the facial nerve (pink arrowhead) and the vestibulocochlear nerve (yellow arrowhead), respectively


A 1-week-old, full-term male newborn presented with increasingly labored breathing, xerostomia, and poor bowel movements

He had been born after an uncomplicated pregnancy, during which routine prenatal ultrasonography had not detected any anatomical abnormalities or lesions

On physical examination, there were no abnormalities other than symmetrical submucosal swelling of the floor of the mouth and a midline external punctum present in the submental area (Figure, A)

The tongue was displaced posteriorly and superiorly

Contrast-enhanced magnetic resonance imaging of the neck and face performed shortly after presentation showed a midline sublingual lesion extending inferiorly through the mylohyoid muscle (Figure, B-D)

The intrinsic muscles of the tongue appeared minimally displaced without obvious infiltration

however, the genioglossus muscle was difficult to locate relative to the mass

A thin-walled, multiloculated lesion was identified, measuring approximately 2.0 × 2.5 × 3.2 cm

The mass was bright on T2-weighted imaging and dark on T1-weighted imaging and did not enhance with intravenous gadolinium

There were no obvious fluid-fluid levels or calcifications

When in-phase and out-of-phase images were compared, there was no significant signal intensity drop, suggesting an absence of fat within the lesion

B, Coronal short tau inversion recovery (STIR) T2-weighted magnetic resonance image without contrast enhancement

C, Coronal T1-weighted, fat-saturated magnetic resonance image after contrast enhancement

D, Sagittal T1-weighted non–fat-saturated magnetic resonance image after contrast enhancement


A teenaged male presented with a 2-week history of vegetating, bleeding plaques and pustules on his face, scalp, trunk, and extremities

He reported that lesions began as pustules and developed into painful, itchy plaques

Before evaluation at a referral hospital in Kenya, the patient was treated with prednisolone tablet 5 mg twice daily, clindamycin hydrochloride 300 mg daily, and fluconazole 200 mg daily, but he continued to develop new lesions

A complete review of systems was notable for the patient’s difficulty with walking because of skin lesions

On examination, pustules and hemorrhagic-crusted plaques were seen on the scalp, legs, arms, and trunk along with keloidal plaques on the face (Figure, A and B)

Enzyme-linked immunosorbent assay testing for HIV was negative

A punch biopsy specimen from the arm was obtained for histopathologic study (Figure, C and D)

Extensive vegetative plaques with bleeding and ulceration on extremities (A), and pustules and ulcerated plaques on extremities and trunk (B)

Histopathologic examination of a biopsy specimen taken from the arm demonstrates pseudoepitheliomatous hyperplasia (hematoxylin-eosin stain, original magnification ×100) (C) and dermal neutrophilia and microabscesses with eosinophilia (hematoxylin-eosin stain, original magnification ×400) (D)


A 26-year-old man presented with a several-year history of persistent round lesions with a scar-like appearance on his face

Before evaluation, his condition had been diagnosed as lupus and lipoatrophy and he tried several treatments with no success

The results of his physical examination showed several round atrophic lesions on his face, symmetric and bilateral, that were located especially on the malar and preauricular areas, with a diameter of 5 to 10 mm

His skin lesions were not indurated and displayed no epidermal changes (Figure 1)

The patient denied experiencing previous inflammation

He had no history of chicken pox or acne or traumatic lesions in those areas

New punch biopsy specimens were obtained for further evaluation

Atrophic lesions on the patient’s left cheek (A) and with a cicatricial appearance on the preauricular right area (B)


Primary penile B-cell non-Hodgkin lymphoma

Primary nodular amyloidosis of the glans penis

A 60-year-old man with a history of IgA kappa multiple myeloma was referred to our department

He had been diagnosed with multiple myeloma 2 years earlier after discovery of osteolytic bone lesions of the skull and the findings of a bone marrow biopsy

The patient had been treated with bortezomib and dexamethasone followed by autologous blood progenitor cell transplantation, which resulted in complete response

At the time of the consultation, he was free of disease

The patient was referred for evaluation of an indolent, erythematous papule on his glans penis of 2 weeks’ duration

He denied sexual risk behavior and reported no recent changes in medication

Careful physical examination revealed a shiny, tense, erythematous, hemispherical papule measuring 5 mm in diameter located on the lateral aspect of the glans (Figure 1A)

The lymph nodes were not palpable, and there was no organomegaly

A punch biopsy was performed for histopathological evaluation (Figure 1B and C)

A, Shiny, erythematous, hemispherical papule on the lateral aspect of the glans penis

B and C, Hematoxylin-eosin stain was performed for evaluation


Amyand hernia (appendix included within the inguinal hernia sac)

A 55-year-old man with a history of Childs B cirrhosis presented with a 2.5-year history of right groin pain and increased urinary frequency with difficulty voiding

On examination, he had a 5 cm × 3 cm palpable and partially reducible right groin mass that extended into the scrotum

He had a leukocytosis with a leukocyte count of 16 400/μL (to convert to ×109/L, multiply by 0.001), his creatinine level was 1.14 mg/dL (to convert to micromoles per liter, multiply by 88.4), and urinalysis showed leukocyturia with cultures that had positive results for Streptococcus viridians

Axial and sagittal (Figure) computed tomography scans with contrast are shown

A renography using mercaptoacetyltriglycine showed a 34% reduction of right renal function

Computed tomography cystogram without intravenous contrast, axial (A) and sagittal (B) view


A healthy 29-year-old pregnant woman, with 34 weeks’ gestational age, presented to the emergency department with diffused acute abdominal pain and vomiting

The pain was located in the periumbilical region and intensified within a few hours

The patient felt normal fetal movements during this time

An initial surgical evaluation was done, and the cause for the abdominal pain was assumed to be not of surgical condition

Next, the patient was evaluated by an obstetrician-gynecologist, and although the gynecologic examination, ultrasonography, and monitoring were all normal, the patient was admitted for further supervision in the obstetrics department

The next day, when the abdominal pain and vomiting persisted and an elevated leukocyte blood count of 14 200/mL was noted (to convert to ×109 per liter, multiply by 0.001), a second surgical consultation was done

The physical examination was significant for diffuse abdominal tenderness

Although the patient was pregnant, and owing to nonavailability of magnetic resonance imaging at that time, a contrast-enhanced abdominopelvic computed tomography was performed (Figure 1)

Coronal computed tomographic image showing an edematous small intestinal loop on the left abdomen, with uterus and head of fetus in the pelvis


Janeway lesions secondary to endocarditis

A black man in his 30s presented for evaluation of papules and plaques on his bilateral lower extremities

Nine months before the current presentation, he was treated with intramuscular penicillin for syphilis

His medical history was also significant for HIV, for which he was receiving highly active antiretroviral therapy, and metastatic programmed cell death ligand 1–positive squamous non–small cell lung cancer

Four months before the current presentation, the patient had started pembrolizumab monotherapy as a first-line treatment for his cancer

One month before this presentation, multiple ulcers developed on his buccal mucosa, as well as a nonpainful shallow penile ulcer and pruritic, mildly painful, hyperpigmented papules and plaques on the bilateral soles of his feet in a pattern identical to previous syphilitic eruption

The patient denied fever, chills, and weight loss

Physical examination revealed multiple hyperpigmented papules and plaques with violaceous borders on his bilateral soles and lateral feet (Figure, A) and scattered, violaceous papules and nodules with overlying scale on his bilateral lower extremities

He was referred to the dermatology service, and a punch biopsy specimen was obtained from the right medial sole (Figure, B)

A, Right foot with multiple hyperpigmented, lichenified papules, and plaques with violaceous borders

B, A punch biopsy specimen from the right medial sole

Lymphocytes, eosinophils, and plasma cells can be seen within the infiltrate (hematoxylin-eosin, original magnification ×40)


A man in his 50s with a history of chronic hepatitis C, hypothyroidism, and alcoholism presented to the outpatient clinic complaining of nasal congestion and epistaxis

Nonenhanced computed tomography of the paranasal sinuses revealed a soft-tissue mass extending from the nasopharynx into the left sphenoid sinus with erosion of the sphenoid sinus floor and the clivus (Figure 1A)

The sella was apparently normal with no disruption of its floor

The intracranial and orbital soft tissues were grossly normal, as were the remaining soft tissues under the skull base

A, Sagittal nonenhanced computed tomography images of the paranasal sinus depicting a soft-tissue mass involving the sphenoid sinus, the nasopharynx, and the clivus

B, Postcontrast sagittal T1-weighted magnetic resonance shows avid enhancement of the mass

involvement of the clivus is also depicted

A magnetic resonance imaging study was also performed (Figure 1B)

There was a lobulated mass centered to the nasopharynx, measuring about 2.8 × 2.6 × 4.5 cm (anteroposterior × transverse × craniocaudal planes, respectively) in maximal diameters

The mass demonstrated intermediate T2 hyperintense signal and avid postcontrast enhancement

It extended inferiorly down to the oropharynx and superiorly along the floor of the left sphenoid sinus with extension into the sinus itself

posteriorly, there were erosions involving the sphenoid bone and the clivus

The floor of sella was intact, and a normal enhancing pituitary gland and infundibulum were seen within the sella

No sellar or suprasellar mass was evident

Both Meckel caves were normal, and the cavernous sinuses were also normal and without evidence of asymmetric mass or abnormal enhancement

The patient underwent a total mass resection via endoscopic anterior cranial base approach, with extradural tumor resection, reconstruction of the skull base, and a nasoseptal flap


A woman in her 30s presented with a 1-year history of left ear fullness

She reported diminished left-sided hearing that would temporarily improve with autoinsufflation

She denied facial numbness or weakness, and reported no dizziness or vertigo

Physical examination revealed a retrotympanic, nonpulsatile, tan-gray mass anterior to the malleus handle

A tuning fork examination revealed lateralization to the left ear and bone conduction louder than air conduction on the left side with a 512-Hz tuning fork

The remainder of the physical examination, including facial nerve function, was normal

An audiogram confirmed mild conductive hearing loss in the left ear

Computed tomography (CT) revealed a middle ear lesion lateral to the geniculate ganglion with possible extension into the peritubal space

Magnetic resonance imaging (MRI) demonstrated an enhancing lesion lateral to the geniculate ganglion (Figure, A-C)

The patient was initially observed but then experienced progressive hearing loss that no longer improved with the Valsalva maneuver, persistent ear fullness, and rare facial twitching

The middle ear mass appeared enlarged on repeated MRI and began to obstruct the eustachian tube

Transcanal endoscopy demonstrated a gray middle ear mass occupying the anterior mesotympanum and epitympanum and extending into the eustachian tube (Figure, D)

The specimen revealed predominantly spindled stroma with a focus of tumor that was positive for antiepithelial membrane antigen and somatostatin receptor 2A

A, Noncontrast axial computed tomogram (CT) of the left temporal bone demonstrates an anterior mesotympanic mass lateral to the cochlea (yellow arrowhead) with blockage and extension into the supratubal recess (white arrowhead)

A partial mastoid effusion (asterisk) is also visible

B, The coronal CT demonstrates an intact tegmen tympani with hyperostosis (arrowhead)

C, Axial, fat-suppressed, postgadolinium, T1-weighted magnetic resonance image (MRI) reveals an enhancing mass of the left perigeniculate region (yellow arrowhead)

The geniculate ganglion is marked (white arrowhead)

D, Intraoperative transcanal photograph of a lobulated, gray, middle ear mass involving the left protympanum and epitympanum


A newborn twin girl was referred to the pediatric otolaryngology clinic at a tertiary pediatric hospital for evaluation of a left-sided nasal mass causing nasal obstruction and difficulty breathing, especially with feeding (Figure 1A)

She was born at 36 weeks’ gestation and spent 10 days in the neonatal intensive care unit

Her parents noted that the lesion was present at birth and she always seemed congested on the left side

Physical examination showed an approximately 1-cm, firm, pedunculated polypoid mass in the anterior nasal cavity at the vestibule

The base was located just anterior to the septum and encompassed nearly the entire left nasal cavity

There was no fluid in the mass, and there was a negative Furstenberg sign

Nasal endoscopy was performed in the clinic, and there were no additional masses or mucosal abnormalities

The patient was also noted to have left eye ptosis and left preauricular branchial remnants but no other significant abnormalities on examination

Magnetic resonance imaging was performed (Figure 1B) and showed a 0.8 × 0.5–cm hyperintense mass on T1-weighted images, suggesting a fatty component and no intracranial tract

Photograph (A) and magnetic resonance imaging (MRI) (B) of the left anterior nasal cavity mass


A 6-year-old boy presented with several tumoral lesions on sun-exposed skin

These lesions developed from local freckles beginning at 6 months of age and gradually spread with protruding growth

Over the past 2 years, tumors started to form and develop rapidly, becoming ulcerative and bleeding

The patient was intellectually standard and denied blurred vision, impaired hearing, or any other systemic symptoms

His parents were first cousins, but no similar cutaneous presentation was reported by other relatives

The physical examination revealed scaly skin with diffuse warty lesions on sun-exposed areas

The patient’s face was severely disfigured, with numerous ulcerative, malodorous tumors (Figure 1A)

The general examination findings, including neurological and ophthalmologic tests, were unremarkable

The lesional discharge culture revealed infection with Proteus vulgaris

Punch biopsy of a paranasal mass was performed (Figure 1B)

Craniofacial computed tomography showed heterogenous soft tissue masses located superficially without bony invasion

No signs of cerebral atrophy were noticed

Genetic testing was advised but was declined

A, Preoperative clinical photography revealed a face severely disfigured by diffuse warty lesions and numerous tumors

B, Infiltrating basal cell carcinoma (original magnification ×40)


A man in his 20s presented with rapidly growing, severely painful oral lesions on the left hard palate of 2 months’ duration

His medical history was significant for Philadelphia chromosome-negative B-cell acute lymphocytic leukemia refractory to multiple treatments, including chimeric antigen receptor T-cell therapy, with transformation to acute myeloid leukemia (AML) status posttreatment

At the time of evaluation, he had been hospitalized for 5 months with severe protein-calorie malnutrition, pancytopenia, and recurrent neutropenic fevers

The patient was febrile (38.3°C), with a white blood cell count of 0.5/μL (absolute neutrophil count 0.00

to convert to ×109/L, multiply by 0.001), hemoglobin levels of 7.6g/dL (to convert to g/L, multiply by 10.0), hematocrit 21%, and platelet count 9/μL (to convert to ×109/L, multiply by 1.0)

He was not receiving active cancer treatment

Intraoral examination revealed poor oral hygiene

The left hard palate exhibited a large swelling with a 5×3-cm indurated area of ulceration, central necrosis, and desquamation (Figure, A)

The patient was treated palliatively with dexamethasone solution and nystatin rinse

A biopsy was not recommended owing to medical instability

However, the patient retained a sloughed fragment of the affected tissue, which was submitted for histologic evaluation

A maxillofacial computed tomography (CT) scan completed the etiological investigation

A, The left hard palate shows enlargement with a large, focal, necrotic lesion

B, Hematoxylin-eosin stain of palatal tissue (original magnification ×1000)

C, Grocott methenamine silver stain of palatal tissue (original magnification ×1000)

D, Computed tomographic image of the maxillofacial complex shows tissue enlargement in the left hard palate and maxillary sinus


A child presented with multiple, asymptomatic, scattered white macules on the lower abdomen and pubic area, which were present at birth and had gradually increased in size and number

Physical examination revealed multiple, well-demarcated, hypopigmented 1- to 5-mm macules on the lower abdomen and pubic area (Figure, A and B)

There was no history of any systemic disorder and no family history of similar lesions

Direct microscopic examination of scales soaked in potassium hydroxide did not reveal hyphae

Wood light examination revealed no accentuation

A punch biopsy specimen was obtained for further evaluation (Figure, C and D)

A, Multiple discrete hypopigmented macules on the lower abdomen and pubic area

B, Well-demarcated hypopigmented macules on the lower abdomen

C, Histopathological examination revealed mild hyperkeratosis, acanthosis, and scattered clear cells in the epidermis (hematoxylin-eosin)

D, The clear cells are larger than the neighboring keratinocytes and contain abundant pale cytoplasm (hematoxylin-eosin)


Extranodal natural killer/T-cell lymphoma, nasal type

Granulomatosis with polyangiitis (Wegener granulomatosis)

A 33-year-old white man presented with a 5-month history of a progressively necrotic plaque on the nose and scattered nodules on the extremities

The plaque first appeared as a small inflammatory papule on the left nostril

Shortly thereafter, the patient was hospitalized at an outside facility for multifocal pneumonia

He was also treated for a methicillin-resistant Staphylococcus aureus infection of his nose

Despite antibiotic therapy, the papule continued to grow and ulcerate and eventually became necrotic (Figure, A)

One month before presentation at our facility, the patient began developing erythematous papules on his arms and legs

Some of them healed spontaneously with poxlike scars

At admission, the patient was tachycardic and febrile to a temperature of 38.5°C

Routine laboratory testing revealed normocytic anemia (hemoglobin, 7.7 g/dL [to convert to grams per liter, multiply by 10])

Shave biopsies of necrotic nodules on the left arm and left cheek (Figure, B-D) and, later, an excisional biopsy of a cervical lymph node were performed

Head computed tomography demonstrated a soft-tissue mass that encased the nasal bone and extended into the right maxillary sinus and left nasal cavity

Chest computed tomography showed bilateral pulmonary nodules

Physical examination revealed a necrotic plaque with surrounding erythema on the distal nose (Figure, A)

A 1 × 1-cm crateriform papule with central necrosis was present on the left malar cheek

Multiple nodules with central, dry necrosis and areas of poxlike scarring were scattered across the extremities

Slight contractures of his fingers were also noted

B-D, Analyses of a shave biopsy specimen of the necrotic papule on the patient’s left cheek (B, hematoxylin-eosin, original magnification ×400


A man in his 50s of West African descent with a history of prostate cancer treated with robotic prostatectomy 2 years ago presented with 1 year of progressively worsening postprandial nausea or vomiting, epigastric abdominal pain, anorexia, and weight loss of 27 kg

Four months earlier, he had presented to another hospital and received a diagnosis of adhesive small bowel obstruction, for which he underwent laparotomy and lysis of small adhesive bands that provided only temporary symptomatic improvement

At his current presentation, he was unable to tolerate any oral intake and entirely dependent on total parenteral nutrition

On examination, he was cachectic with a moderately distended, nontender abdomen

The result of a blood test (QuantiFERON-TB Gold

Qiagen) was positive for tuberculosis exposure

An abdominal computed tomography scan showed markedly dilated stomach and duodenum with decompressed, thickened distal small bowel without a clear transition point

Nonoperative management with persistently high nasogastric tube output failed, and the patient was subsequently taken to the operating room for exploratory laparotomy

Intraoperative findings are shown in Figure 1.Exploratory laparotomy showing the appearance of the small bowel


Posterior perforation of a duodenal ulcer

Infected aortic graft with aortoduodenal fistula

A 74-year-old man presented to the primary care physician with a 3-month history of weight loss, anorexia, and back pain

His medical history was significant for hypertension, hyperlipidemia, and kidney stones

His surgical history was significant for a bilateral staged carotid endarterectomy in 1999 and 2000 and an open repair of a 7.4-cm transverse diameter juxtarenal abdominal aortic aneurysm with a knitted Dacron tube graft (18 mm) in 2014. Ligation of the left renal vein adjacent to the inferior vena cava was performed to gain proximal control of the aorta

The patient underwent extraction of a tooth secondary to an abscess 4 months earlier

Physical examination revealed poor dental hygiene and findings in the lower extremity suggestive of postthrombotic syndrome

The patient was afebrile with a heart rate of 108 beats per minute and a leukocyte level of 11 600/cm (normal range, 4000-11 000/cm), with 84% polymorphic neutrophils (normal, <75%)

erythrocyte sedimentation rate was 57 mm/h (normal range, 0-10 mm/h), and C-reactive protein level was 15.9 mg/dL (normal, <0.6 mg/dL [to convert to nmol/L, multiply by 9.524])

Computed tomography (CT) of the abdomen and pelvis was performed (Figure 1A and B)


A 67-year-old man presented with a rapidly enlarging scalp lesion (Figure 1A) first noticed 3 months earlier as a pimple

The lesion was itchy but without generalized pruritus

The patient reported a weight loss of 10 pounds over the preceding 3 months

In addition, he reported a longer history of vague, nonspecific, intermittent abdominal pain without nausea, vomiting, or fever starting about 3 years prior to presentation

Previous work-up had revealed minimal abdominal adenopathy, but the patient declined further workup

On examination, he had no other cutaneous lesions, but he did have cervical lymphadenopathy

All other physical findings were within normal limits

Laboratory findings were unremarkable, save for a mild normochromic normocytic anemia and a mildly elevated levels of lactate dehydrogenase

A biopsy specimen from the skin lesion is shown in Figure 1B

A, Clinical image of the patient at presentation

B, Biopsy specimen from the skin lesion is shown


A woman in her early 70s presented to her local emergency department with the recent onset of wheezing and dyspnea

Noncontrast computed tomography of her chest showed a 1.1-cm nodular lesion in the upper trachea that appeared to be originating from the right lobe of the thyroid (Figure 1A)

She was referred to our center for evaluation and management of what appeared to be an aggressive thyroid malignant neoplasm

She presented with obvious biphasic stridor and a report of dyspnea on mild exertion

A CT of the chest showed a soft-tissue mass protruding into the trachea in the posterolateral aspect of the right side of the tracheal region that appeared to be contiguous with the thyroid gland

Ultrasound-guided fine-needle aspiration of the thyroid was performed

however, there was no nodule in the right thyroid lobe that correlated with the CT findings

The left thyroid lobe contained an 8-mm nodule

Examination of a biopsy specimen from this nodule showed medullary thyroid cancer (MTC)

The patient’s serum calcitonin level was 23 pg/mL

Magnetic resonance imaging (MRI) of the neck revealed a hyperintense tracheal mass on T2-weighted imaging (Figure 1B) that showed enhancement on administration of contrast medium (not shown)

In addition, a 0.3-cm nonenhancing nodule at the lower pole of the right thyroid lobe, separate from the tracheal mass, was identified

Vocal cord mobility was normal on fiberoptic laryngoscopy, and the patient verbally denied having dysphagia

Both ultrasonography and CT/magnetic resonance imaging failed to identify any suspicious adenopathy

A, Axial computed tomogram (CT) of the chest without contrast medium shows a 1.1-cm hypodense, exophytic right posterolateral intratracheal mass with apparent continuity with the right thyroid lobe

B, Axial, T2-weighted magnetic resonance image (MRI) with contrast medium revealed a hyperintense soft-tissue mass within the tracheal lumen

A T1-weighted image before the addition of contrast medium (not shown) showed a slightly hyperintense mass to the thyroid gland

That lesion showed enhancement on administration of contrast medium


A woman in her 70s with a history of pulmonary embolism presented to the emergency department with acute right eye vision loss

She described a 2-week medical history of diplopia and blurry vision as well as 4 months of nasal congestion and progressive headaches despite multiple antibiotic courses

She denied a medical history of sinonasal issues, immunodeficiency, autoimmune disease, malignant disease, or diabetes

On examination, she was afebrile with stable vital signs

Ophthalmologic evaluation revealed a right afferent pupillary defect and cranial sixth-nerve palsy

Computed tomographic and magnetic resonance imaging of the sinuses revealed abnormal soft tissue throughout her sinuses with destruction of the sinus walls and skull base as well as extension of abnormal appearing tissue into the right orbital apex and posterior nasal septum

Laboratory evaluation demonstrated a normal white blood cell count and an elevated erythrocyte sedimentation rate and C-reactive protein level

Nasal endoscopy showed bloody mucus and crusting around the right middle turbinate and sphenoethmoidal recess

The acute nature of her vision loss amid concern for a destructive sinonasal process warranted operative intervention

Abnormal soft tissue with associated destruction throughout her sinonasal cavities and right orbital apex was identified

Frozen sections revealed negative results for fungal elements and cellular dysplasia but demonstrated widespread inflammation with giant cells

A representative final pathology slide is shown in the Figure

Sinonasal mucosal biopsy results demonstrate numerous multinucleated giant cells (black arrowheads), necrosis (white arrowhead), and inflammatory cells invading vessel walls (blue arrowheads)

Hematoxylin-eosin stain (original magnification ×10)


Granulomatosis with polyangiitis (Wegner granulomatosis) and relapsing polychondritis

A 32-year-old Hispanic man without significant medical history presented with nasal pain and swelling, progressive for 6 weeks

He had been hospitalized for this problem at an outside hospital and treated with antibiotics, without improvement

The swelling worsened and extended to his upper lip

He developed difficulty swallowing, headache, and nasal drainage

On presentation, he was afebrile without leukocytosis

Physical examination demonstrated diffuse swelling and tenderness of the nose with honey-colored crusting that extended to above his upper lip

Axial and sagittal contrast-enhanced computed tomography (CT) (Figure, A and B) demonstrated marked soft tissue swelling and fullness with abnormal enhancement along the nasal cavity, including bilateral nasal ala, with areas of necrosis and emphysema

No significant bony erosion or associated calcification on bone windows was noted

After bedside debridement of the nasal soft tissue region, axial postcontrast T1-weighted fat-suppressed and sagittal T2-weighted fat-suppressed magnetic resonance imaging through the nasal cavity (Figure, C and D) demonstrated soft tissue fullness isointense to muscle on T1-weighted images, hyperintense on T2-weighted images, and with homogenous enhancement of the solid component on postcontrast T1-weighted images

No intracranial or intraorbital disease extension was noted

Contrast-enhanced computed tomography (CT) demonstrates marked soft tissue swelling and fullness with abnormal enhancement along the nasal cavity, including bilateral nasal ala, with areas of necrosis and emphysema

Fat-suppressed magnetic resonance imaging (MRI) demonstrates soft tissue fullness isointense to muscle on T1-weighted images, hyperintense on T2-weighted images, and with homogenous enhancement of the solid component on postcontrast T1-weighted images


A girl presented with a left posterior triangle neck mass that had progressed for 3 years following a motor vehicle accident

On physical examination, the mass was firm, mobile, tender to palpation, and caused restricted range of motion

Ultrasound imaging of the soft tissue of the neck was obtained, which demonstrated a neck mass with dense acoustic shadowing (Figure 1A)

Further evaluation with contrasted computed tomography (CT) imaging showed a 3.3×2.9×2.6-cm ossified lesion in the left posterior-lateral cervical soft tissue near the atlanto-axial junction

The lesion was well circumscribed and separate from the underlying bone (Figure 1B)

The patient underwent excision of the mass without complication

The gross pathology specimen showed a firm, irregular, encapsulated mass

A, Ultrasonography of the neck soft tissue demonstrating a neck mass with dense acoustic shadowing in the left posterior triangle

B, Axial computed tomographic imaging of the neck with contrast depicting a 3.3-cm well-circumscribed ossified mass that is separated from the C2 vertebral body

No aggressive-appearing periosteal reaction is present (arrowhead indicates calcified neck mass)


Familial benign pemphigus (Hailey-Hailey disease)

A man in his 40s presented with a 3-month history of discrete, dark red, circular lesions in his right axilla

He endorsed severe pruritus at these sites that would wake him up at night

He denied any recent trauma or history of similar lesions

He denied associated pain, blisters, or systemic symptoms (eg, fever, chills)

His medical history was unremarkable, and he noted no environmental triggers or new medications

Treatment with over-the-counter hydrocortisone cream, ketoconazole cream, and discontinuation of underarm deodorant yielded no improvement

Physical examination revealed 3 well-circumscribed, violaceous, annular patches without scale or erosion that are limited to his right axilla (Figure, A)

No other lesions were noted on his skin or mucous membranes

A punch biopsy specimen from a lesion was obtained (Figure, B and C)

A, Clinical photograph shows oval and annular thin violaceous patches in the right axilla

B, Hematoxylin-eosin stain (original magnification ×40) demonstrates lymphocytes obscuring the dermal-epidermal junction

C, Hematoxylin-eosin stain (original magnification ×200)


An 80-year-old white woman presented with an asymptomatic white patch affecting the tongue of 1 month’s duration

She had a 15-year history of oral lichen planus (OLP) managed with betamethasone dipropionate, 0.05%, gel twice daily and clotrimazole troches, 10 mg, 3 times daily as needed for symptomatic OLP flares

Medical history revealed stage 1A mycosis fungoides/cutaneous T-cell lymphoma affecting the right calf and left thigh managed primarily with halobetasol, 0.05%, cream owing to intolerance of narrowband UV-B therapy and mechlorethamine, 0.016%, gel

Immunosuppression or history of infectious diseases, including human immunodeficiency virus (HIV), was not reported

A 1.5 × 1.0-cm nonremovable white, plaquelike lesion was observed on the left lateral tongue (Figure 1A)

Biopsy specimens were obtained with a 3-mm punch instrument at 3 different sites, which demonstrated similar microscopic findings (Figure 1B and C)

A, A corrugated, nonremovable white lesion on left lateral tongue

B, Histologic image showing hyperkeratotic, acanthotic stratified squamous epithelium exhibiting cells with lightly stained cytoplasm (balloon cells) throughout the stratum spinosum and superficial layer (hematoxylin-eosin)

C, Histologic image showing several epithelial nuclei with peripheral chromatin margination also described as chromatin beading (arrowheads) (hematoxylin-eosin)


Pigmented squamous cell carcinoma in situ

A man in his 40s presented to the dermatology clinic for evaluation of a new pigmented lesion on his left second toenail

The lesion first appeared 12 months before presentation and was progressively growing

This was his first time presenting to a physician for this problem, and no prior treatments had been attempted

He was otherwise well and denied any fevers or weight loss

Clinical examination revealed a dark brown longitudinal pigment band covering the full length of the toenail of the left second digit with corresponding nail thickening over the pigmented band

The pigment was the same width throughout the band and extended to the proximal nail fold (Figure, A)

No other nail or skin lesions were found at the time of examination

A biopsy specimen was obtained from the nail plate and nail matrix and stained with hematoxylin-eosin (Figure, B-D)

A dark brown longitudinal pigment band on the second toenail with pigment extends to the proximal nail fold (A)

Histopathologic examination of the specimen with hematoxylin-eosin stain is seen at original magnification of ×40 (B), ×100 (C), and ×200 (D)


Autosomal recessive spastic ataxia of Charlevoix-Saguenay

Complicated hereditary spastic paraplegia

A 16-year-old girl presented to our ataxia unit with a report of a peculiar finding in fundoscopy

Her parents were nonconsanguineous, and her family history was unremarkable

She had unsteadiness and frequent falls at gait initiation since age 16 months

There was a progressive worsening of her gait difficulties, and she complained of loss of balance

On examination, she had saccadic pursuit, subtle appendicular ataxia, and a spastic gait with a wide base

She could not stand or walk with her feet in the tandem position

Achilles tendon reflexes and plantar responses were absent, and vibration sense was decreased in her toes and ankles

Visual acuity, color vision, and visual fields were normal

Fundoscopy revealed increased visibility of retinal nerve fibers, hiding the contours of retinal vessels bilaterally

Optical coherence tomography (OCT) showed increased thickness in the retinal nerve fiber layer (RNFL) (Figure 1)

Ophthalmological findings include color fundus photograph of the right eye (A) with increased visibility of the retinal nerve fiber layer, which hides retinal vessel contour

an optical coherence tomographic vertical B-scan revealing a markedly thickened retinal nerve fiber layer (asterisks) (B), captured along the green line seen on a red-free image of fundus (C)


Posterior lateral muscular atrophy with pseudohernia

Superior lumbar hernia (Grynfeltt-Lesshaft hernia)

A 65-year-old woman was seen at the surgical clinic with reports of chronic back pain and a bulge in her back

History revealed multilevel cervical and lumbar fusion surgical procedures using anterior, left lateral, and posterior approaches over a 10-year period

The patient subsequently noted a new, slowly enlarging bulge in her left midback that was bothersome with activities of daily living (Figure, A)

She denied pain directly overlying the area and was otherwise tolerating a regular diet and having usual bowel function

Physical examination revealed a soft bulge in the lateral left midback

There were no apparent associated skin changes, and the palpable mass appeared fixed and nonreducible

The mass was most apparent with upright posture and was accentuated with a cough impulse, but it was less distinct when examined in the prone position

Computed tomography of the abdomen and pelvis was obtained (Figure, B)

Patient presentation (A) and computed tomography of the abdomen and pelvis (B)


Thyroid goiter with compressive symptoms

A 56-year-old man presented to the emergency department with a 3-week history of worsening neck and shoulder pain

He had been seen in his primary care physician’s office about 2 weeks prior to emergency department presentation and had been treated for musculoskeletal symptoms

He admitted to paresthesia and pain radiating down his right arm but denied any weakness

He had no difficulty with speech or swallowing

He had no medical comorbidities or prior surgery

On examination, he was noted to have some cervical midline spine tenderness

No weakness or sensory deficits were identified

No palpable lymphadenopathy, swelling, or mass was noted

A plain radiograph of the neck demonstrated tracheal deviation, and he then underwent computed tomography of the chest (Figure 1A) and magnetic resonance imaging of the cervical and thoracic spine (Figure 1B)

Computed tomography scan and magnetic resonance image

A, Coronal computed tomography image of the chest

B, sagittal magnetic resonance image of the cervical and thoracic spine


Primary malignant mediastinal germ cell tumor

Nuclear protein in testis midline carcinoma

A young man in his early 20s presented with a 1-day history of pleuritic chest pain and shortness of breath, with no fever or cough

He smoked approximately 10 cigarettes per day for 4 years

Results of the physical examination were unremarkable

Radiography of the chest revealed an ill-defined consolidation in the right lower lung field

Laboratory investigations were significant for elevated levels of lactate dehydrogenase (273 U/L

reference range, 135-225 U/L [to convert to microkatals per liter, multiply by 0.0167]), C-reactive protein (125.3 mg/L

reference range, 0.0-4.9 mg/L [to convert to nanomoles per liter, multiply by 9.524]), and alkaline phosphatase (217 U/L

reference range, 40-129 U/L [to convert to microkatals per liter, multiply by 0.0167])

Computed tomography of the chest confirmed the presence of a dense consolidation involving most of the right middle lobe with right hilar and subcarinal lymphadenopathy (Figure, A)

He underwent endobronchial ultrasonography-guided transbronchial needle aspiration of a fleshy, whitish endobronchial lesion obstructing both uptakes to the medial and lateral segments

Histologic analysis revealed a poorly differentiated malignant neoplasm (Figure, B)

Most of the cells were relatively monotonous, with enlarged vesicular nuclei, prominent nucleoli, and a high ratio of nucleus to cytoplasm

Foci of abrupt squamous differentiation, including keratinization and intercellular bridges, were identified

Immunohistochemical staining showed tumor cells to be strongly and diffusely positive for keratin AE1/AE3, cytokeratin 5/6, and p63. However, findings were negative for S-100, melan A, placental alkaline phosphatase, CD56, thyroid transcription factor-1, terminal deoxynucleotidyl transferase, CD45, and cytokeratin 7. A positron emission tomography–computed tomography scan showed a fludeoxyglucose-enhanced avid right middle lobe mass, a large right-sided pleural effusion, and fludeoxyglucose-enhanced avid mediastinal and right hilar lymph nodes, with extensive skeletal metastases to both proximal humeri and scapulae, the sacrum, spine, iliac bones, and both hips

A, Computed tomography of the chest revealed a dense consolidation in the middle lobe of the right lung with right hilar and subcarinal lymphadenopathy

B, Histologic analysis of the mass revealed a poorly differentiated malignant neoplasm

Most cells showed enlarged vesicular nuclei, prominent nucleoli, and a high ratio of nucleus to cytoplasm (hematoxylin-eosin


A cigarette smoker in his 50s with a history of hypertension, chronic kidney disease, colon adenocarcinoma, and hyperuricemia presented with a right vocal fold mass that had been an incidental finding during esophagogastroduodenoscopy

He denied any symptoms related to the lesion, including dysphonia or dysphagia, and reported no fevers or weight loss

Rigid stroboscopy revealed normal vocal fold mobility and a nodular cystic-appearing lesion on the posterior inferior border of the right vocal process (Figure, A)

There was no overlying ulceration, and vocal fold mucosal wave was intact in the adjacent membranous vocal fold

Suspension microlaryngoscopy incisional biopsy of the right infraglottic lesion was performed in the operating room

The endotracheal tube was displaced anteriorly with the laryngoscope

Intraoperatively, there was submucosal white caseous debris in the substance of the lesion

The surgical specimen was sent for histopathologic analysis (Figure, B)

Cultures were analyzed for the presence of fungus, bacteria, and mycobacteria

Postoperatively, the patient was doing well and continued to have no symptoms of dysphagia or dysphonia


Angiolymphoid hyperplasia with eosinophilia

A farmer in his 50s presented with bilateral periauricular swelling that began 6 months prior (Figure 1)

The swelling was mild at onset and gradually progressive

There was no history of associated pain, but he reported occasional pruritus over the swelling

There was no history of fever, weight loss, or loss of appetite

He denied any history of similar swelling elsewhere in his body

General physical and systemic examination had normal results

On examination of the swelling on the right side, there was a diffuse swelling around the pinna measuring approximately 6 cm over the preauricular, postauricular, and infra-auricular region

It was a nontender, soft swelling without fluctuation and abnormal pulsations

There were similar findings in the left side

Mouth opening was adequate, secretions from both parotid ducts were normal, and facial nerve function was intact on both sides

He had undergone fine-needle aspiration cytology and incisional biopsy

The results of both were consistent with reactive lymphadenitis

He was prescribed oral antibiotics for 1 week but showed no improvement

A contrast-enhanced computed tomographic scan of the head and neck showed bilateral multiple enhancing lymph nodes in the postauricular region, the largest measuring 16 × 11 mm2 on the right side and 15.4 × 10.6 mm2 on the left, along with bilateral intraparotid nodes, the largest measuring 16.4 × 10.2 mm2 on the right and 15.3 × 10.7 mm2 on the left side

His serum eosinophil count was slightly elevated, and his erythrocyte sedimentation rate was 15 mm/h

He was then scheduled for excisional biopsy at our center

The biopsy specimen included skin and subcutaneous tissue along with 2 underlying lymph nodes

Clinical photographs showing diffuse swelling of the preauricular, infra-auricular, and postauricular region


A man in his 40s with Poland syndrome presented with a 6-month history of left throat pain and enlarging oropharyngeal mass

The patient had been seen repeatedly for odynophagia that persisted despite multiple courses of antibiotics, reflux medications, and steroids prescribed at clinics and emergency departments

Over the course of 5 months, he underwent a tonsillectomy and 3 separate biopsies at multiple institutions

All showed inconclusive results, and the mass progressively enlarged

At presentation for the present report, physical examination showed a 2-cm, well-mucosalized, left oropharyngeal mass not extending to the hard palate (Figure, A)

Computed tomography (CT) revealed a 58-mm parapharyngeal mass lateralizing and narrowing the left carotid artery against the styloid process (Figure, B)

A CT-guided fine-needle aspiration biopsy was performed but again was nondiagnostic

Further confounding the clinical picture, a pacemaker was required for multiple episodes of sinus asystole due to mass effect on the carotid body

To obtain the diagnosis of the parapharyngeal mass, the patient had a transcervical resection with transoral partial glossectomy, palatectomy, and pharyngectomy with reconstruction

Results of immunohistochemical analysis of the removed tissue were positive for CD4 (weak), CD21, CD23, and vimentin with a low proliferation rate (Figure, C and D)

A, A 2-cm well-mucosalized left oropharyngeal soft-palate mass anterior to the tonsillar bed with surrounding erythema

B, Left parapharyngeal mass measuring 4.3 × 2.7 × 5.8 cm and lateralizing the left carotid artery

C, Fascicular, storiformed architecture with spindly, polygonal-shaped cells with eosinophilic cytoplasm and indistinct borders (original magnification ×10)

original magnification ×10) and CD23 (right


A woman in her 60s presented for initial dermatologic evaluation of a 2-year history of a pruritic and sometimes burning hyperpigmented eruption on the bilateral palms

The eruption had gradually spread from the left palm to the right palm and then focally onto the bilateral volar aspect of the wrists in recent months (Figure, A and B)

She had not experienced involvement of the plantar surfaces

No additional concerns were reported, and there was no family history of similar lesions

There was no known history of travel in relation to onset of the lesions

Limited clinical examination revealed well-marginated brown atrophic patches on the bilateral palms extending focally onto the volar aspect of the wrist and centrally surmounted by spiny keratotic pits in the palmar creases

Two punch biopsy specimens were obtained for diagnostic clarification (Figure, C and D)

A, An atrophic hyperpigmented plaque with spiny papules in the palmar creases

B, Similar features extending onto the volar wrist

C and D, Histopathologic images (hematoxylin-eosin, original magnification ×100)


A woman in her 70s presented with a 1-year history of a facial cutaneous eruption initially affecting malar cheeks and eyebrows

Subsequently it spread to involve nose, chin, upper trunk, and extremities

Her medical history included a cadaveric renal transplant 2 years earlier for end-stage renal failure

Immunosuppressant medications included mycophenolate mofetil, 200 mg, twice daily and tacrolimus, 8 mg, twice daily

Physical examination revealed multiple 1-mm flesh-colored follicular papules and keratin spines against a diffuse erythematous background affecting the face (Figure, A and B) and upper trunk

Her scalp and eyebrow hairs were unremarkable

Results of skin biopsy from the right ear demonstrated dilatation and keratotic plugging of the hair infundibula with marked dystrophy and expansion of the inner root sheath

The inner root sheath cells were enlarged with irregular trichohyaline granules and apoptotic cells with abrupt cornification without formation of a granular layer (Figure, C)

Immunohistochemical analysis for SV40 polyomavirus was positive (Figure, D)

Photographs of (A) forehead and (B) nose depicting multiple follicular papules and keratin spines against a diffuse erythematous background

C, Hematoxylin-eosin staining (original magnification ×40)

D, SV40 polyomavirus stain (original magnification ×40)


Erythema papulosa semicircularis recidivans

A healthy woman in her 50s presented with a large annular erythematous plaque on her lower back that had appeared in June (1 month previously) and had centrifugally expanded

She complained about mild pruritus that had not responded to conventional antihistamine therapy or topical steroids

She could not recall an arthropod bite prior to the skin eruption and denied having traveled outside Spain in the past few years

She reported a 20-year history of the same annular plaques recurring on the trunk every single year by the beginning of summer and spontaneously resolving by the beginning of autumn

Such lesions had never appeared on the face, hands, or feet

She did not relate these lesions to any particular hobby or application of any skin care product

Physical examination revealed a large, 35-cm semicircular erythematous plaque on her lower back with central clearing and a distinctive, well-demarcated papular border (Figure, A and B)

After obtaining informed consent, 2 biopsy specimens were taken from the papular border of the plaque for both hematoxylin-eosin stain and direct immunofluorescence (Figure, C)

A, Large erythematous semicircular plaque with central postinflammatory hyperpigmentation on the lower back

B, Absence of epidermal changes, mild edema of the papillary dermis, and a superficial sparse perivascular inflammatory infiltrate

C, Inflammatory infiltrate composed mainly by lymphocytes and few neutrophils

D, Follow-up visit showing clinical resolution in October


A 63-year-old man with a history of well-controlled hypertension, lumbar stenosis, and hip arthroplasty with revisions presented with bilateral loss of vision and hearing for several weeks

He also reported a history of painful paresthesias at his feet beginning about 1 year prior

On examination, his visual acuity was initially 20/80 OD and 20/200 OS

it deteriorated over the next 2 months to finger counting bilaterally

The Weber test gave a nonlateralizing result, and bone conduction hearing was absent bilaterally

There was length-dependent loss of vibratory sensation and absent reflexes at both lower extremities

Magnetic resonance imaging demonstrated abnormal enhancement of bilateral optic nerves (Figure, A)

Optical coherence tomography and fluorescein angiogram were normal

Nerve conduction studies showed mild sensory axonal polyneuropathy

Laboratory tests were notable for elevated thyrotropin (63 mlU/ml), low levels of free thyroxine (T4) (0.2 ng/dL), and elevated levels of cerebrospinal fluid protein (94 mg/dL)

Cerebrospinal fluid IgG index, oligoclonal bands, aquaporin 4 antibody, paraneoplastic panel, and protein electrophoresis test results were all normal

He was treated empirically with high-dose steroids and plasmapheresis, with no improvement in symptoms

A, Axial postgadolinium, fat-suppressed, T-1–weighted magnetic resonance image of the orbits, demonstrating left greater than right enhancement of the optic nerves (arrowheads)

The enhancement is centrally located within the optic nerves on coronal sections (not shown)

B, Axial T-2–weighted magnetic resonance image of the right hip, demonstrating susceptibility artifact of orthopedic hardware, as well as soft tissue heterotopic ossification (corroborated by plain-film radiographs), consistent with pseudotumor of metallosis with decompression into the right hip joint space (blue asterisk), the subiliac bursa (red asterisk), greater trochanter bursa (yellow asterisk) extending into the gluteus maximus muscle (arrowheads), and into a potential space between the tensor fascia lata and the rectus femoris (black asterisk)

Four months after initial presentation, the patient reported new symptoms of chest tightness and dyspnea on exertion

An echocardiogram showed a left ventricle ejection fraction of 20% to 25%, which had been 55% to 60% at the last test 2 years prior

At 5 months after initial presentation, the patient developed acute worsening of dyspnea and was admitted for respiratory distress

He was found to have nonischemic cardiomyopathy and died on hospital day 13.


A woman in her early 50s with a history of polycystic ovarian syndrome, diabetes, stroke, and coronary artery disease that required coronary artery bypass graft 6 years earlier presented to the emergency department with 12 hours of sudden-onset, constant, right lower quadrant abdominal pain associated with vomiting and melena

She denied any hematochezia or history of peptic ulcer disease

She had no other surgical history and denied alcohol use or smoking

On examination, she was hypertensive (blood pressure, 185/85 mm Hg) although not tachycardic and was afebrile

She was locally tender to palpation in the right lower quadrant with rebound and voluntary guarding

The patient had no abdominal scars, hernias, or palpable masses

Laboratory results were significant for the following: leukocyte count, 23 600/µL (to convert to ×109/L, multiply by 0.001) with neutrophilia (94%)

blood urea nitrogen level, 28 mg/dL (to convert to millimoles per liter, multiply by 0.357)

creatinine concentration, 0.01 mg/dL (to convert to micromoles per liter, multiply by 88.4)

lipase concentration, 195 U/L (to convert to microkatals per liter, multiply by 0.0167)

and lactate concentration, 2.02 mg/dL (to convert to millimoles per liter, multiply by 0.111)

Electrolyte levels and liver function test results were within normal limits

An electrocardiogram showed normal sinus rhythm

Computed tomography of the abdomen and pelvis with oral and intravenous contrast enhancement was performed (Figure)

Axial cross-sectional computed tomograms of the patient’s abdomen and pelvis with oral and intravenous contrast enhancement show segmental mural thickening of the anterior cecum with wall hypoenhancement and pneumatosis, adjacent fat stranding, and a lucency medial to the cecum (A) and bilateral renal hypodensities (B)


Rupture of an infected endometriotic implant

Malignant growth with unknown primary tumor

A postmenopausal woman in her mid-50s (gravida 2, aborta 2) with a known history of endometriosis and adenomyosis presented to the emergency department with severe abdominal pain that she rated 10 of 10 in intensity and which had developed suddenly 5 days before presentation

The abdominal pain was associated with diarrhea, nausea, and abdominal bloating

On examination, the patient was afebrile and her abdomen was soft and nondistended without guarding or rebound but tender in the left lower quadrant

The patient had a palpable nodule in the posterior vaginal fornix and fullness in the left lower quadrant of the abdomen on bimanual examination

Laboratory investigations had normal findings with the exception of an elevated cancer antigen 125 level of 158 U/mL (to convert to kilounits per liter, multiply by 1.0)

Contrast-enhanced computed tomography (CT) revealed multiple abnormal findings that included a bulky uterus with a thickened myometrium, endometriomas, right pleural effusion, pelvic peritonitis and ascites, enlarged lymph nodes, and solid extracolonic implants in the omentum caudal to the transverse colon (Figure, A) and in the cul-de-sac adjacent to the upper rectum (Figure, B)

Subsequent magnetic resonance imaging (MRI) showed 2 areas of focus corresponding with the extracolonic implants


On day 15 of induction therapy with intravenous cytarabine, vincristine sulfate, dexamethasone, pegaspargase, and intrathecal methotrexate, a 3-year-old girl with B-cell acute lymphoblastic leukemia presented to the oncology department with an asymptomatic skin lesion

The patient was afebrile, and vital signs were stable

On examination, a 4.0 × 2.0-cm, oval-shaped violaceous to black plaque with a central hemorrhagic bulla and surrounding hyperemia was noted on the left lower back (Figure 1)

A complete blood cell count revealed a white blood cell count of 1800/μL (to convert to ×109/L, multiply by 0.001), with 2% neutrophils (absolute neutrophil count, 36/μL [to convert to ×109/L, multiply by 0.001]), hemoglobin level of 8.2 g/dL (to convert to grams per liter, multiply by 10), and platelet count of 10 × 103/μL (to convert to ×109/L, multiply by 1)

Her blood glucose level was 326 mg/dL (to convert to millimoles per liter, multiply by 0.0555)

The patient was administered broad-spectrum antibiotics empirically, and a dermatologist was consulted

Two punch biopsy specimens from the lower back plaque were obtained and sent for histologic analysis and tissue culture (bacteria, atypical mycobacteria, and fungus)

Violaceous to black plaque with central hemorrhagic bulla on the left lower back of the patient


A woman in her 80s presented to hospital with somnolence and fatigue of 2 days’ duration

Medical history included lower urinary tract symptoms of 1-week duration for which she was prescribed cotrimoxazole with no improvement

She also reported progressively worsening vulvar and buttock pain of 5 days’ duration

The patient was admitted to the intensive care unit 3 weeks prior to presentation for pneumonia and remained hospitalized for 10 days

She was not sexually active and had no medical history of sexually transmitted infections

Anogenital examination revealed vulvar (Figure 1A) and buttock ulcers (Figure 1B) tender to palpation

Initial blood workup showed leukopenia with a white blood cell count of 2400 cells/μL (to convert to cells/L, multiply by 106), a differential of 72% neutrophils, and a hemoglobin level of 8.5 g/dL with a hematocrit differential of 27% (to convert hemoglobin to g/L, multiply by 10.0)

Cotrimoxazole was discontinued on admission

A biopsy specimen was obtained from the left vulvar ulcer (Figure 1C)

A and B, Clinical photographs showing vulvar and buttock ulcers at the time of presentation

C, Hematoxylin-eosin stain (original magnification ×40)


A 21-year-old woman with no significant medical history presented with a 2-year history of asymptomatic, facial redness that flared in sunlight

She had been previously treated with doxycycline, 100 mg/d, and topical sulfacetamide with no effect

On examination, on the cheeks and nasal bridge there were multiple erythematous, annular plaques with focal areas of atrophy

Her medications included only an oral birth control pill

Serum chemical analyses and complete blood cell count showed no abnormalities, and anti-Ro, anti-La, and qualitative ANA antibodies were negative

A clinical diagnosis was made, and over the next 9 months, treatments with minocycline, desonide, topical metronidazole, 0.75%, cream and azelaic acid, 15%, were unsuccessful, and the plaques developed more scarring and atrophy (Figure, A)

In addition, the patient began to develop a burning sensation in these areas

A punch biopsy specimen was obtained and submitted for histopathologic review (Figure, B and C)


A young child presented to clinic with a 6-month history of an asymptomatic expanding erythematous eruption on the lower legs, abdomen, and buttocks (Figure, A)

The eruption would wax and wane, with each episode lasting for approximately 1 week, without residual pigmentation

Clinical examination was significant for faint, nonscaling annular serpiginous, erythematous plaques with central clearing, and barely elevated borders

Test results for erythrocyte sedimentation rate, complete blood cell count, antinuclear antibodies, anti-Ro/SSA and anti-La/SSB antibodies, and Borrelia burgdorferi antibodies were within normal limits

There was no family history of autoimmune disease, and the child had not experienced any fevers or exposures to ticks or other arthropods

A punch biopsy specimen from the left thigh was obtained, and histopathological analysis was subsequently performed (Figure, B and C)

A, Annular serpiginous plaques with areas of central clearing on the patient’s right thigh and buttocks

B and C, Histopathologic images (hematoxylin-eosin)

B, Perivascular and inflammatory infiltrate

C, Perivascular and inflammatory infiltrate that includes eosinophils