A 7-month-old infant presented to the otolaryngology clinic for noisy breathing

His noisy breathing had been present since birth, constant, exacerbated with feeding, sleeping, and supine positioning

He fed poorly from the bottle with frequent choking, grunting, and irritability, and had minimal weight gain with 1 cyanotic event while feeding

His medical history was significant for prematurity, born at 26 weeks, and a 60-day intubation during a 99-day neonatal intensive care unit stay

The physical examination was remarkable only for biphasic stridor and a weak cry

Nasopharyngolaryngoscopy showed no laryngomalacia, laryngeal lesions, or cord paralysis

Shortly after this visit, the patient presented to the emergency department with increased work of breathing, tachypnea, retractions, weak cry, and stridor

Examination was significant for subcostal retractions, and biphasic stridor

Due to a lack of response to racemic epinephrine, albuterol, and steroids, he was admitted to the intensive care unit for heliox and intravenous steroids, resulting in mild improvements

A viral panel was positive for rhinovirus

A few days later he was taken to the operating room for direct laryngoscopy and bronchoscopy

During sedation, a prolonged expiratory phase with quick chest wall rise on inspiration and slow fall with expiration was observed

Capnography showed a restrictive “shark fin” waveform (Figure, A)

The direct laryngoscopy and bronchoscopy were performed (Figure, C and D)

Images show the (A) preintervention and (B) postintervention capnography wave form as seen on the anesthesia monitor

Endoscopy images on (C) expiration and (D) inspiration


A man in his 60s presented with a 3-month history of intertrigo involving his groin that he had been treating with a combination of clotrimazole cream, 1%, and hydrocortisone cream, 1%

His intertrigo improved, but he then developed bright red, burning and painful nodules around his scrotum (Figure 1)

His medical history included prostate cancer, hypertension, anxiety disorder, hyperlipidemia, gastroesophageal reflux disease, and diverticulosis

His medications included omeprazole, alprazolam, amlodipine besylate, atenolol, simvastatin, and valsartan

On physical examination, there were 2 symmetrical solitary erythematous nodules in the right and left inguinal folds adjacent to the scrotum

Clinical image of well-demarcated erythematous solitary papule in the right inguinal fold


A previously healthy man in his 30s was referred to our department for evaluation of asymptomatic cutaneous lesions that had been present for 4 months

He had been treated with a course of high-potency topical corticosteroids without any improvement

Physical examination revealed annular red plaques with scale and superficial erosions over the trunk and extremities

Strikingly, plaques on both arms showed a corymbiform pattern, with a central and multiple satellite lesions (Figure, A)

On the lower limbs, infiltrated plaques with prominent crusts were seen (Figure, B)

Also, there were papulosquamous lesions on the left sole, but the palms were spared

There was neither regional lymph node enlargement nor systemic involvement

Two biopsy specimens from both types of lesions were obtained for histopathologic evaluation, showing an identical histologic pattern (Figure, C and D)


A 40-year-old man of Scandinavian descent presented with enlarging, nonpainful upper extremity nodules developing over the preceding 9 months (Figure, A)

He was renovating a commercial cannabis grow-house in California at time of onset

He described the construction site as being filled with intoxicating mold

He reported shortness of breath and a 20-pound weight loss

He denied recent travel or aquarium exposure

Most concerning was the inability to use his right upper extremity

Also, he developed a pathological fracture of his right foot (Figure, B)

Clinical examination of his forearms revealed several yam-size (4-6 cm), nontender, nonulcerated, boggy subcutaneous nodules in a sporotrichoid pattern and large nodules scattered on his trunk and abdomen

There was no palpable lymphadenopathy or lower extremity lesions

His medical history is significant for diabetes mellitus type 2 and hypothyroidism

Medications included glipizide, metformin, and levothyroxine

A, Nodules are 4 to 6 cm in diameter on the right upper extremity

B, A pathological fracture on the fifth metatarsal on the right foot is indicated by the white arrowhead

C, Histopathology shows pale, nodular aggregations of inflammatory cells in the deep dermis, extending into the fibrotic subcutis, as well as (D) aggregations composed of epitheliod histiocytes with few other inflammatory cells, so called “naked granulomas

”The patient’s laboratory tests showed fasting glucose of 122.0 mg/dL (reference range, 65.0-115.0 mg/dL)

total calcium, 10.2 mg/dL (reference range, 8.5-10.5 mg/dL)

blood urea nitrogen, 16.0 mg/dL (reference range, 10.0-24.0 mg/dL)

creatinine, 1.30 mg/dL (reference range, 0.6-1.3 mg/dL)

hemoglobin A1c, 6.7 (reference range, 4.0-6.0)

1,25-OH vitamin D 70, ng/mL (reference range, 15.9-55.6)

Liver enzymes were within normal limits along with a negative purified protein derivative and quantiferon test results

A culture from a cutaneous nodule with microbial stains for bacteria, fungal culture, and acid-fast bacilli was negative

Computed tomography of the chest revealed hilar lymphadenopathy

An incisional biopsy of a nodule on the right wrist was obtained, which showed nodular aggregations of epithelioid histiocytes with sparse peripheral lymphocytes, extending from the deep dermis into the subcutis (Figure C and D)

Periodic acid–Schiff diastase and Fite stains were negative for fungal and mycobacterial organisms, respectively


An 11-year-old boy of East-Asian descent presented to the hospital with an 8-month history of progressive vision loss, night blindness, and light sensitivity

He also had a long-standing complaint of dry eyes

His medical history included diagnoses of eczema and multiple food allergies

Owing to concerns about food triggers of his eczema, the child had a restrictive diet consisting solely of potato, pork, lamb, apples, cucumber, and Cheerios

There was no history of redness, discharge, or trauma to the eyes

The vision loss was not associated with pain, headache, fever, or photopsias (flashes of light)

On examination, the child’s visual acuity was limited to detecting hand motion at 30 cm

His conjunctiva had dry, keratinized patches with foamy appearance (Figure 1A)

The cornea showed areas of marked epitheliopathy with opacity (Figure 1B)

His pupils were equal and reactive to light with no relative afferent pupillary defect

His funduscopic examination showed mild optic nerve pallor

A, Photograph of the patient’s eye before treatment

Arrowheads indicate dry keratinized patches (Bitot spots)

B, Photograph of the patient’s eye before treatment demonstrating marked corneal epitheliopathy with opacity


A 48-year-old man presented to the emergency department with a 1-week history of worsening right lower quadrant tenderness and fullness

This was associated with diarrhea, vomiting, fevers, and chills

He reported some relief of symptoms with loperamide

He denied any history of weight loss, melena, or hematochezia

Other than a previous open hemorrhoidectomy, the patient had no significant medical history

Of note, he had no family history of intestinal disease

On examination, he had a fever with a temperature of 39°C and tachycardia with a heart rate of 102 beats per minute

On abdominal examination, there was localized tenderness with guarding and fullness in the right lower quadrant

Results from his blood laboratory investigations demonstrated leukocytosis, with white blood cell count of 17 000/µL (to convert to ×109/L, multiple by 0.001) and a C-reactive protein level of 205 mg/L (to convert to nmol/L, multiple by 9.524)

However, given the clinical history, the patient underwent a computed tomographic (CT) scan of the abdomen and pelvis with intravenous contrast (Figure 1)

He then underwent an exploratory laparotomy

Transverse computed tomographic scan indicated a large right-sided inflammatory phlegmon measuring 7.5 cm in transverse diameter in a 48-year-old man


A woman in her 50s with a history of systemic lupus erythematosus, congestive heart failure, and type 2 diabetes presented to the emergency department with worsening abdominal pain 1 day after discharge from an admission for similar abdominal pain

The patient described a 4-day history of progressively worsening abdominal pain and diarrhea and denied fevers, chills, diaphoresis, or any previous similar episodes

Her abdomen was distended and mildly tender, and her pain was disproportionate to the findings of her physical examination

There were no abnormal laboratory test findings

The abdominal computed tomography (CT) scan from her initial admission (Figure, A) and the abdominal CT angiogram on readmission (Figure, B) showed dramatic progression of her disease

A, Coronal view of computed tomography (CT) scan from 3 days prior to readmission demonstrating isolated duodenal thickening

B, coronal view of CT angiography scan on readmission demonstrating thickened small bowel and free fluid


Fournier gangrene (necrotizing fasciitis of the perineum)

Scrotal ulceration secondary to induction chemotherapy with ATRA

A previously healthy man in his 30s presented with spontaneous bruising, epistaxis, and haematuria

Acute promyelocytic leukemia (APML) was diagnosed by bone marrow aspirate morphology, immunophenotyping, and detection of the bcr1 variant of the PML-RARα fusion transcript in peripheral blood

He was commenced on treatment with the PETHEMA LPA 20051 protocol consisting of all-trans retinoic acid (ATRA) and idarubicin, an anthracycline based chemotherapy

Dexamethasone was prescribed to reduce the risk of ATRA syndrome, a potentially life threatening complication seen in these patients and characterized by fever, pulmonary infiltrates, hypotension, and leucocytosis

After 12 days of treatment, the patient developed pyrexia and was started on empiric antibiotics

Two days later he developed tender black spots on the scrotum that progressed to painful ulcers over 8 days (Figure, A)

Systemic antifungal and antiviral therapy were added with no improvement

Peripheral blood cultures and skin swabs from the lesions did not identify bacterial or viral infection

Scrotal ultrasonography showed a subcutaneous hypervascular lesion with no abscess

The development and progression of scrotal ulceration coincided with recovery in neutrophil count from 0.3 × 109/L to 1.2 × 109/L and persistent pyrexia

As scrotal ulceration progressed, they enlarged and became necrotic with a black eschar

A specimen was obtained for histopathology

A, Clinical photograph of scrotum and penis shows ulceration and necrosis of penile and scrotal skin with surrounding inflammation

B, Scrotal biopsy histopathologic specimen (hematoxylin-eosin


A woman in her 30s with a 1-year history of unintentional weight loss, mild diffuse abdominal pain, and occasional episodes of vomiting presented to the emergency department with a 24-hour history of intense abdominal pain, fever, and loose stools

An abdominal computed tomographic scan showed findings consistent with bowel perforation, and she was admitted to the intensive care unit after emergent exploratory laparotomy

Her medical history was significant for 1 other episode of idiopathic bowel perforation 3 years prior

Dermatology was consulted to evaluate an asymptomatic skin eruption that had been present for at least 8 months before her hospitalization

Physical examination revealed numerous 5- to 10-mm nontender papules with a porcelain-white atrophic center and an erythematous halo, alongside scattered atrophic white scars (Figure, A and B)

The lesions were distributed across her trunk, neck, and upper and lower extremities

Palms, soles, scalp, genitalia, and mucosal surfaces were spared

Her neurological and mental state examination had unremarkable results

Biopsy specimens from her thigh and back were obtained (Figure, C and D)

Clinical photographs and biopsy specimens

A, Papules on the back with white atrophic center and an erythematous rim

B, Lesions on the thigh similar to those of the back, alongside atrophic white scars

C, Thigh biopsy shows hyperkeratosis and wedge-shaped avascular necrosis in dermis and subcutaneous tissue (hematoxylin-eosin stain, original magnification ×10)

D, Back biopsy shows occlusion of dermal blood vessels, perivascular lymphocytic infiltration, and mucin deposition (hematoxylin-eosin stain, original magnification ×40)


A woman in her 80s presented with a 1-year history of pruritic erythema on the trunk and extremities

The erythema did not respond to topical corticosteroid, oral roxithromycin (300 mg/d), or narrow-band UV-B phototherapy

The patient had a history of type 2 diabetes and hypertension and was treated with glimepiride, sitagliptin phosphate hydrate, and amlodipine besylate

The patient also had polymyalgia, which was treated with a low dose of prednisolone

The prednisolone therapy had been discontinued 2 months before her visit to the dermatology clinic when she had been hospitalized with pneumonia

Multiple tense blisters had subsequently appeared on the palms

Physical examination at the first visit revealed scaly erythematous plaques on the whole body and tense blisters on the arms, hands, soles, and buttocks

Neither scar formation or mucosal involvement was observed

A skin biopsy specimen was obtained from a bulla on the forearm

Histopathologic analysis revealed a subepidermal bulla with inflammatory infiltrates of neutrophils

The results of an IgG chemiluminescence enzyme immunoassay for bullous pemphigoid (BP) 180 noncollagenous (NC) 16A were negative

After treatment with oral prednisolone, 20 mg/d, the lesions healed without scar or milia formation (Figure 1)

Clinical features before (A) and after (B) treatment with oral prednisolone, showing no scar formation


Neonatal-onset multisystem inflammatory disorder

A 5-year-old white girl presented with a history of recurrent fevers and urticaria since 2 months of age

The child had fevers (maximum temperature, 40°C) that lasted 3 to 5 days

Her mother reported that the fevers resolved spontaneously but continued to recur almost every month

The fever symptoms were associated with bilateral conjunctivitis, arthralgias, and irritability

The hivelike, nonpruritic urticaria did not improve with antihistamine medication and there were no known precipitating or aggravating factors

Family history was noncontributory and her parents denied consanguinity

The child was hospitalized on multiple occasions during infancy for the fevers, and she had comprehensive sepsis evaluations, including lumbar punctures, with negative results

Findings from chest and abdominal imaging and bone marrow aspiration with biopsy were negative for a malignant process

Despite extensive evaluation, a definitive source for the fevers was not identified, and the patient was referred to our institution

On physical examination, the patient was febrile (temperature, 39°C) and appeared fussy

Skin examination confirmed nonpruritic, macular and urticarial lesions on the face, back, and lower extremities (Figure)

Bilateral conjunctival hyperemia without drainage was noted

There was neither swelling nor redness of the joints

Her right ankle and knee examination showed limited active and passive range of motion secondary to pain symptoms

Findings from the remainder of physical examination were normal

Erythematous macular and urticarial lesions with areas of confluence on back and lower extremities


Secondary lymphedema of the upper extremity

An elderly woman was transferred from an extended care facility with lower abdominal pain

She was afebrile with a heart rate of 120 beats/min on admission and leukocytosis (white blood cell count, 23 000/μL [to convert to ×109/L, multiply by 0.001])

Computed tomography of the abdomen and pelvis and transvaginal ultrasonography revealed a large intrauterine abscess

An attempt to insert a peripherally inserted central catheter (PICC) into the right basilic vein was unsuccessful

An intravenous catheter in the left hand was established for the administration of antibiotics

The following day, the patient developed right upper extremity pain and swelling

Physical examination revealed tense and tender swelling of the right upper extremity that was more marked in the upper arm and caused significant impairment in motor function, but sensation to touch and pin prick was intact (Figure 1A)

The right radial pulse was absent, but biphasic Doppler arterial signals were present in the right radial and ulnar artery

Duplex ultrasonographic imaging of the right upper extremity demonstrated a 12.8 × 2.6-cm heterogeneous fluid collection with active color flow (Figure 1B)

Patient presentation (A) and ultrasonographic imaging (B) of the upper arm

Extensive deep vein thrombosis of the upper extremityCompartment syndrome resulting from infiltration of intravenous infusion therapy into the soft tissues of the upper extremity


Acute perforation of a bleeding peptic ulcer

Rupture of a peripancreatic pseudoaneurysm

A man in his 50s with a history of smoking and chronic hypertension was admitted to our hospital with a 36-hour history of upper abdominal pain, nausea, and vomiting

He had no history of previous surgery or trauma

His systolic blood pressure was 109 mm Hg, his diastolic blood pressure was 70 mm Hg, and his pulse rate was 100 beats/min

A physical examination revealed moderate epigastric tenderness with rebound and guarding

Laboratory test results were remarkable for a white blood cell count of 13 970/μL, hemoglobin level of 8.4 g/dL, serum creatinine level of 1.67 mg/dL, serum amylase level of 57 U/L (reference range, 30-110 U/L), and serum triglyceride level of 282.42 mg/dL, with no evidence of coagulopathy

(To convert the white blood cell count to ×109 per liter, multiply by 0.001

hemoglobin level to grams per liter, by 10

serum creatinine level to micromoles per liter, by 88.4

serum amylase level to microkatals per liter, by 0.0167

and serum triglyceride level to millimoles per liter, by 0.0113.) Contrast-enhanced computed tomography (CECT) and mesenteric angiography were performed (Figure 1)

A, Contrast-enhanced computed tomographic (CECT) image showing an intra-abdominal hyperdense mass (arrowhead) and free fluid in the upper abdomen

B, Visceral artery angiogram revealing an aneurysm (arrowhead)


Carcinoma erysipeloides (cutaneous metastases of breast cancer)

A woman in her 60s presented with a 3-month history of a progressive, asymptomatic cutaneous eruption on her chest and back

She had a complex history of breast cancer on the left side that was first diagnosed in 1991 and was treated with lumpectomy, radiotherapy, cyclophosphamide, methotrexate, and fluorouracil

In 2001, she experienced local disease recurrence and underwent complete mastectomy with axillary lymph node dissection on the left side and received adjuvant treatment with tamoxifen citrate and anastrozole

In 2014, she developed axillary lymphadenopathy on the right side and was found to have metastatic disease

She subsequently underwent axillary lymph node dissection on the right side, followed by treatment with doxorubicin hydrochloride and cyclophosphamide

Her most recent mammogram and positron-emission tomography–computed tomography (PET/CT) showed no evidence of active disease

At the time of her evaluation, the patient’s physical examination was remarkable for discrete erysipeloid plaques on her chest, back, and right shoulder (Figure 1A)

The lesions were warm and indurated but nontender to palpation

Serum chemistry profile, complete blood cell counts, and hepatic and renal function testing had normal results

Lesional skin biopsies were performed (Figure 1B)

A, Clinical examination shows discrete erysipeloid plaques on the anterior chest

B, Histopathologic analysis (hematoxylin-eosin stain, original magnification ×100)


A healthy woman in her 60s presented with multiple erythematous crusted plaques on her trunk that had appeared 4 weeks previously, sparing her limbs and facial region (Figure, A)

She had a long-standing history of several outbreaks of skin lesions limited to both axillae and groin

Previous episodes had been successfully treated with various topical drugs that were administered by her primary care physician

No family history of similar lesions was recorded

Physical examination revealed multiple annular erythematous plaques surrounded by a peripheral crusted and pustular collarette on her trunk (Figure, B)

No active lesions were present in her axillae, inframammary folds, or groin

After obtaining informed consent, 2 biopsy specimens were taken from the periphery of the lesion for hematoxylin-eosin stain and direct immunofluorescence (Figure, C and D)

A skin swab specimen from a pustular lesion was taken for microbiological culture

A, Multiple erythematous and crusted plaques diffusely distributed on the dorsal trunk at presentation

B, An erythematous annular plaque, surrounded by a crusted and focally pustular peripheral collarette

C and D, Histopathologic images, hematoxylin-eosin

C, Biopsy specimen revealing suprabasilar acantholysis (original magnification ×4)

D, Specimen from generalized lesions at clinical presentation (original magnification ×40)


A white woman in her 50s presented to an oral medicine clinic for evaluation of asymptomatic pigmented lesions affecting the maxillary and mandibular gingiva of 6 years’ duration

The patient reported previous orolabial melanotic macules

Her medical history was significant for hypertension, hypercholesterolemia, asthma, and breast cancer

Medications included levalbuterol, nadolol, and cetirizine

Family medical history was significant for breast cancer and social history was unremarkable

Review of systems was positive for cutaneous ephelides

Physical examination revealed a 7 mm × 6 mm asymmetric, heterogeneously pigmented lesion without indurated borders on the gingiva associated with the left maxillary central incisor tooth (number 9) (Figure, A)

A lesion similar in clinical appearance, although smaller, was observed on the gingiva associated with the right mandibular first premolar tooth (number 28) (Figure, B)

Biopsy specimens were obtained with a 3 mm punch instrument from both lesions for routine histology which demonstrated similar microscopic findings (Figure, C and D)

A, Heterogeneously pigmented lesion of the gingiva associated with the left maxillary central incisor

B, Heterogeneously pigmented lesion of the gingiva associated with the right mandibular first premolar

C, Mucosal epithelium exhibiting spongiosis and a diffuse proliferation of heavily pigmented dendritic melanocytes that extend the full thickness of the epithelium (arrowheads)

Melanin incontinence is also noted (hematoxylin-eosin stain, original magnification ×100)

D, Squamous mucosa exhibiting spongiosis with diffuse pigmentation in the basal cell layer and dendritic melanocytes (arrowheads) in the stratum spinosum (hematoxylin-eosin stain, original magnification ×200)


A female infant with relapsed refractory infantile acute lymphoblastic leukemia, blast crisis (>60% blasts), and central nervous system involvement was admitted for reinduction chemotherapy with cytarabine, vincristine, daunorubicin, methotrexate, and dexamethasone

Seventeen days after her last day of chemotherapy, while neutropenic, she became febrile and developed several vesicles with an erythematous base on the lower back (Figure, A) and left thigh

Tissue and blood cultures and a punch biopsy specimen of a vesicle on the lower back were obtained

Hematoxylin-eosin (Figures, B and C), gram (Figure, D), and periodic acid-Schiff stains were performed on the punch biopsy

Testing for herpes simplex virus and varicella zoster virus were also ordered

A, Scattered vesicles with an erythematous base on the lower back

B, Hematoxylin and eosin stain (original magnification ×200)

C, Hematoxylin and eosin stain (original magnification ×600)

D, Gram stain (original magnification ×600)


A teenage Chinese girl with intermittent limp and increasing headache was admitted to the hospital

She began to limp intermittently ten years earlier

A brain magnetic resonance imaging (MRI) showed multiple parenchymal lesions involving the pons, bilateral amygdala, and the left temporal pole (Figure 1A)

She refused further investigations until a year earlier, when she developed mild headache

Magnetic resonance imaging showed minor focal enlargement of the lesions in the left temporal pole

One month before hospital admission, her headache aggravated and became intolerable

Enhanced MRI showed remarkable progression of the lesions in the left temporal lobe, involving the temporal pole and the parasylvian region, along with a newly developed mass in the third ventricle and obstructive hydrocephalus

The lesions in the pons and the right amygdala remained stable (Figure 1B)

There was no history of tumors elsewhere

Her consciousness was intact, but her memory and concentration were poor

There were multiple congenital nevi distributed over the body, 2 of which on the legs were more than 5 cm in diameter, and another 3 on her back and face were more than 3 cm

There were no focal motor or sensory deficits

The rest of the neurologic and systemic examination was unremarkable

Gene examinations showed a mutation in the N-ras-2 gene

A and B, T1-weighted axial brain magnetic resonance imaging at the levels of pons, midbrain, and thalamus, performed ten years earlier, shows multiple parenchymal lesions including the pons, bilateral amygdala, and the left temporal pole (arrowheads)

contrast, 640. C and D, Enhanced, T1-weighted axial brain magnetic resonance imaging at the levels of pons, midbrain, and thalamus, performed at the most recent hospital admission, shows remarkable progression of the lesions in the left temporal lobe, involving the temporal pole and the parasylvian region, along with a newly developed mass in the third ventricle (arrowhead) and obstructive hydrocephalus


A previously healthy 15-year-old boy presented to our hospital with pain and purpura of his left thigh after an intramuscular injection in that leg

Ten days prior, he had presented to a referring hospital’s emergency department for nausea and vomiting and was given 20 mg of intramuscular dicyclomine

He reported severe pain at the time and site of injection

Within a few hours, he noticed swelling and purpura overlying the site of injection

Over the next 2 weeks, the swelling persisted and the purpura intensified, becoming black centrally (Figure A and B)

Pain worsened over this period, initially restricting use of the leg, then preventing any weight bearing, prompting his admission to our hospital 10 days after the injection

He also reported numbness and occasional tingling of the skin involved

The patient denied fever or chills, and his nausea and vomiting had resolved

A, Teenage boy with development of retiform purpura of the left thigh following intramuscular dicyclomine injection

At 2 days after injection, red to purple retiform changes developed on the left thigh

B, Worsening retiform purpura with central necrosis was noted 13 days after the injection

Findings of a physical examination were significant for retiform purpura of the left thigh, approximately 8 × 12 cm in size

Palpation of the area elicited severe pain

Popliteal, posterior tibial, dorsalis pedis, and femoral pulses were strong

There was no swelling of the remainder of the leg, and findings of joint examinations were normal

Sensation was intact throughout the leg with the exception of the skin involved, which had diminished light touch

Blood test results revealed a normal complete blood cell count, normal coagulation profile, and an elevated creatine phosphokinase level of 477 U/L (normal range, 22-198 U/L

to convert to microkatals per liter, multiply by 0.0167)

Magnetic resonance imaging of the thigh was completed, and a skin biopsy was performed for culture and histology


A previously healthy 4-year-old girl with a history of prematurity (31 weeks’ gestational age) presented as a transfer from an outside facility with abdominal pain

She had presented to an outside emergency department 2 days prior with lower abdominal pain that subsequently localized to the right lower quadrant

Her pain was accompanied by anorexia and nonbloody, nonbilious emesis

She was noted to have voluntary guarding and focal right lower quadrant tenderness to palpation

Laboratory analyses demonstrated a mild leukocytosis (white blood cell count = 14 × 103/μL [to convert to ×109/L, multiply by 0.001]) and normal urinalysis results

Abdominal ultrasonography images were obtained that failed to show her appendix and demonstrated no secondary signs of inflammation

Because of her notable tenderness, she was admitted for observation at the referring facility

No antibiotics were initiated, and her activity improved with intravenous hydration

However, her right lower abdominal pain persisted, prompting further evaluation

Computed tomography images of the abdomen and pelvis were obtained, and the representative images are shown in Figure 1. She was transferred to our facility for surgical evaluation

In our emergency department, pelvic ultrasonography images were obtained that failed to show both the appendix and right adnexal structures

Axial (A) and (B) coronal sections demonstrate an inflamed retrocecal mass


A 62-year-old woman presented to the emergency department with severe substernal pain radiating to the left shoulder and back and subsequent swelling of the left side of her neck

She had been recently treated at an outpatient facility for a viral illness with multiple episodes of forceful coughing

There was no recent history of trauma to the neck or chest

Physical examination revealed normal vital signs and soft tissue swelling over the left supraclavicular area

No overlying skin changes or crepitus were appreciated

Laboratory data revealed leukocytosis (white blood cell count, 18 000/µL

to convert to ×109 per liter, multiply by 0.001)

A computed tomographic scan demonstrated extensive edema of the left neck, with fat stranding seen in the subcutaneous tissues extending into the left jugular, carotid, and esophageal spaces (Figure, A)

Mediastinal edema and bilateral pleural effusions were also seen on computed tomographic scan (Figure, B)

Owing to the concern for esophageal perforation, a barium swallow was performed, showing no extravasation of contrast

A, Computed tomographic scan shows extensive soft tissue inflammation in the left neck (arrowhead)

B, Computed tomographic scan shows bilateral pleural effusions (red arrowheads) and mediastinal edema (white arrowhead)


A woman in her 60s presented for evaluation of multiple painful cutaneous nodules that had developed over the previous 2 months

The patient had a history of IgGκ multiple myeloma, diagnosed approximately 6 months earlier

At that time, flow cytometry showed a κ-restricted plasma cell population positive for CD38 and CD138 but negative for CD3, CD20, and CD45 expression

Cytogenetic analysis with fluorescent in situ hybridization did not reveal high-risk genetic markers

The patient demonstrated no evidence of end organ disease, and her myeloma was classified as stage II

Since her initial diagnosis, she had completed 4 cycles of lenalidomide, bortezomib, and dexamethasone (RVD) therapy in addition to irradiation for lytic lesions of the thoracic and lumbar spine

Physical examination revealed scattered red-violaceous nodules, with the largest measuring approximately 6.0 × 5.5 × 2.5 cm (Figure, A)

They were found in the mouth, the trunk, and on all 4 extremities

The lesions were nonulcerated, round with a smooth surface, fixed, and firm and tender to touch

An ultrasound-guided needle biopsy and immunohistochemical analysis of the largest lesion on the patient’s right shoulder were performed (Figure, B and C)


A man in his 50s with a history of both hypertension and a pulmonary tumor that was resected 10 years earlier (Figure, A) presented with generalized phymatous changes affecting his entire face, as well as a few pustular lesions and a diffuse erythema that gave him an appearance of leonine facies (Figure, B)

He complained of substantial, progressive seborrheic hyperplasia and flushing episodes over the past year, accompanied by ocular manifestations that included swelling of his eyelids

Ophthalmological examination showed features of chronic blepharitis and lagophthalmos

Detailed anamnesis revealed a history of intense diarrhea in prior months

A biopsy specimen from the skin of the forehead was obtained and analyzed under hematoxylin-eosin staining (Figure, C)

A computed tomography (CT) scan completed the etiological investigation (Figure, D)

A, Immunohistochemical analysis of the earlier lung tumor using chromogranin A

panoramic view shows that most of the cells diffusely express chromogranin (original magnification ×20)

B, Leonine facies presentation shows phymatous changes, diffuse erythema, pustular lesions, and seborrheic hyperplasia

C, Forehead skin shows scarce lymphocytic infiltrate close to follicular structure along with sebaceous hyperplasia (hematoxylin-eosin, original magnification ×40)

D, Liver computed tomographic (CT) image showing multiple metastatic hepatic masses


A woman in her 50s presented for evaluation of itching skin lesions

The lesions had been present for months and increased gradually

Treatment with topical steroids showed no lasting effect

Skin examination revealed multiple flaccid pustules on scaly, erythematous plaques on her trunk, with an annular and circinate pattern (Figure, A and B)

A and B, Clinical presentation showed flaccid pustules on scaly, erythematous plaques on the trunk

C, Subcorneal acantholysis of keratinocytes (yellow arrowheads) resulting in intraepidermal separation/blistering and an inflammatory infiltrate consisting of neutrophilic granulocytes (black arrowheads) (hematoxylin-eosin staining, original magnification × 100

D, Discrete subcorneal intercellular IgA depositions (white arrowheads) (direct immunofluorescence with FITC-labeled antihuman IgA antibodies, original magnification × 400)


A 4-year-old girl with newly diagnosed acute lymphocytic leukemia, receiving her initial course of chemotherapy with vincristine, daunorubicin, and methotrexate, developed a painful lesion on her left triceps

The tender area was beneath an adhesive bandage placed where she had received DTaP (diphtheria-tetanus-pertussis) and polio vaccinations 3 weeks earlier

Otherwise, she appeared remarkably well, and her vital signs were normal

Removing the bandage revealed a tender indurated gray-violet plaque (Figure, A)

White blood cell and neutrophil counts were low at 0.4 K/μL (normal range, 4.86-13.18 K/μL) and 0.18 K/μL (normal range, 1.60-8.29 K/μL), respectively

Punch biopsy specimens were obtained for histopathologic analysis (Figure, B) and fungal culture (Figure, C)

A, Gray-violet plaque on posterior upper arm at site of adhesive portion of bandage

B, Broad nonseptate hyphae surrounding and infiltrating dermal blood vessels (hematoxylin-eosin

Of note, occasional septae are seen in this specimen, consistent with the characteristic finding that mucormycetes are pauciseptate, rather than aseptate, molds

C, Nonseptate hyphae with round terminal sporangia and free spores (lactophenol cotton blue mount


A 3-day-old premature male infant, born at 26 weeks’ gestational age, presented to the dermatology department with a skin rash on the bilateral lower extremities

History was notable for preterm premature rupture of membranes for 10 days

The neonate was born via urgent cesarean delivery owing to breech positioning

The mother was otherwise in good health with normal prenatal care and had no history of sexually transmitted diseases

During the first few days of life, the neonate had recurrent hypoxic episodes

He was receiving intravenous antibiotics for suspected sepsis and bacitracin for the topical lesions

Per the primary team, the rash was first noted at 3 days of life

Physical examination demonstrated a small, ill-appearing neonate with multiple, grouped vesicles and punched-out ulcerations on his scalp, axilla, extremities, and scrotum (Figure, A-D)

He also had witnessed respiratory desaturations requiring urgent intubation

Initial laboratory evaluation demonstrated a normal complete blood cell count and serum chemistry with the exception of a high lymphocyte count (34%, normal level <25% [to convert to proportion of 1.0, multiply by 0.01]) and high aspartate aminotransferase level (117 IU/L, normal level 8-39 IU/L  [to convert to microkatals per liter, multiply by 0.0167])

A chest radiograph demonstrated increased pulmonary markings bilaterally

Neonate with grouped vesicles on an erythematous base on the scalp and bilateral lower extremities


A 28-year-old woman visited the emergency department for a sudden, diffuse, and painful enlargement of the upper quadrants of the left breast with edema and palpable axillary lymph nodes without clinical signs of inflammation or infection (Figure 1A)

She had neither fever nor skin redness but was experiencing fatigue

A, Asymmetric mass in the upper quadrant of the left breast

B, Breast ultrasonogram revealing hypoechogenicity of the breast tissue with periductal small cysts

The patient had stopped breastfeeding 10 months before the emergency department admission, and she had a recent history of pneumonia self-medicated with antibiotics

She had no family history of breast or ovarian cancer and no history of drug addiction or hyaluronic acid or free silicone injections in the breast

On physical examination, the patient had a 6-cm hard mass without nipple discharge or retraction and left axillary palpable lymph nodes

Breast ultrasonography revealed diffuse nonspecific hypoechogenicity of the breast tissue with periductal small cysts (Figure 1B)

Mammography revealed asymmetric density and distortion in the left upper quadrants

Multiple 14-gauge ultrasound-guided biopsies in the upper quadrants of the left breast and fine-needle aspiration cytologic testing of an enlarged lymph node revealed granulomatous inflammation with epithelioid histiocytes, lymphocytes, plasma cells, and eosinophils

Blood test results were as follows: hepatitis B surface antigen, hepatitis C antibody, human immunodeficiency virus enzyme-linked immunosorbent assay, and whole-blood interferon γ release assay (QuantiFERON), negative

fibrinogen level, 234 mg/dL (to convert to micromoles per liter, multiply by 0.0294)

leukocyte count, 7660/µL (to convert to ×109/L, multiply by 0.001)


A 69-year-old white man presented to the emergency department after a tractor-on-tree collision

Contrast-enhanced computed tomography demonstrated an incidental right infrarenal, retroperitoneal mass measuring 9.7 × 7.7 × 9.1 cm (Figure 1A), with dilated tortuous supply from a lumbar artery branch and retroperitoneal lymphadenopathy

He denied any history of systemic symptoms, pain, urinary symptoms, or abnormal bowel function but described 6 years of decreased appetite

Laboratory evaluation of plasma-free metanephrine levels showed a slight elevation in normetanephrine levels (1.21 nmol/L)

Given the dramatic arterial enhancement and hypertrophied perivascular plexus, biopsy was not performed, and preoperative arterial embolization was deemed appropriate to minimize intraoperative blood loss

A, Contrast-enhanced computed tomography scan demonstrating an incidental right infrarenal, retroperitoneal mass measuring 9.7 × 7.7 × 9.1 cm, with dilated tortuous supply from a lumbar artery branch

B, Flush aortogram outlining supply to the mass from a hypertrophied lumbar artery (blue arrowhead) and from a branch of the internal iliac artery (red arrowhead)

At preoperative embolization, initial aortography identified the predominant lumbar artery branch and also demonstrated supply from a branch of the internal iliac artery (Figure 1B)

These branches were embolized and the mass was devascularized

Twenty-four hours following embolization, surgical exploration was performed through a periumbilical midline incision

With no evidence of distant metastasis (peritoneal or liver), the mass was resected with partial resection of the iliopsoas muscle to achieve negative margins in conjunction with aortocaval lymphadenectomy of the enlarged lymph nodes

The mass was running parallel to the right ureter, which was meticulously dissected, and the right kidney was successfully saved

There were no complications, and the postoperative course was uneventful


A man in his 60s presented with progressive onset of nocturnal dyspnea, dysphonia, dysphagia, and noisy breathing over the previous year

In addition, he reported a 22.7-kg weight loss

He also had a medical history significant for obstructive sleep apnea, gastroesophageal reflux disease with Barrett esophagitis, diabetes, and degenerative joint disease

Findings from his head and neck physical examination were normal except for the laryngeal examination

Laryngeal endoscopy revealed a large, well-mucosalized mass emanating from the left pyriform sinus, with mass effect on the larynx (Figure, A)

The bilateral true vocal folds (TVFs) were visualized and were normal, but the full range of mobility of the left TVF could not be assessed owing to mass effect

A computed tomographic scan with contrast of the neck was obtained and revealed a 5 × 6-cm, nonenhancing, well-circumscribed laryngeal mass (Figure, B)

The tumor was originating from the left pyriform sinus and did not appear to be locally invasive

Given these findings, the patient underwent direct laryngoscopy for transoral carbon dioxide laser–assisted excision without complications

Microscopic examination of the mass demonstrated polygonal cells with peripherally placed nuclei and eosinophilic cytoplasm containing cross-striations (Figure, C)

Postoperatively, the patient reported improvement in dysphagia, voice quality, sleep, and breathing patterns

A, A large left pyriform sinus mass with mass effect on the larynx

B, Preoperative computerized tomography scan showing a large laryngeal mass without local invasion

C, Polygonal cells with peripherally placed nuclei and eosinophilic cytoplasm containing cross-striations (hematoxylin-eosin, original magnification ×40)


A man in his 40s with newly diagnosed acute myelogenous leukemia (AML) arising from myelodysplasia presented with fever, cough, abdominal pain, difficulty swallowing, and 1 month of neutropenia

He had abruptly developed a painful eruption on his scalp, face, and left leg 24 hours prior to presentation after being scratched in these areas by his pet cat

The patient had not received any chemotherapy or antimicrobial prophylaxis

Physical examination revealed an ill-appearing, diaphoretic, thin man with multiple erythematous to violaceous, tender, edematous, targetoid nodules and plaques with central dusky appearance on the left forearm and right upper thigh (Figure, A)

Vital signs included temperature of 39.7° C, blood pressure of 141/69 mm Hg, and pulse of 122 bpm

His white blood cell count was 1.8 × 103/µL (reference range, 4.5-11 × 103/µL

to convert to ×109/L, multiply by .001), and absolute neutrophil count was 246 cells/µL (reference range, 1520-6370 cells/µL)

Other laboratory values were remarkable for a red blood cell count of 2.13 × 106/µL (reference range, 3.9-5.5 × 106/µL

to convert to ×1012/L, multiply by 1), hemoglobin of 6.1 g/dL (reference range, 14-17.5 g/dL

to convert to g/L, multiply by 10), and platelet count of 14 × 103/µL (reference range 150-350 × 103/µL, to convert to ×109/L, multiply 1)

Cellular cytogenetics of his AML were notable for a 5q deletion and p53 mutation

Punch biopsy specimens from the right arm and left thigh were performed for histopathologic evaluation and tissue culture

A brisk neutrophilic infiltrate was seen on histopathology (Figure, B)

A, 1.5 cm-long, edematous plaque with dusky central erythema on upper thigh

B, Punch biopsy specimen with hematoxylin-eosin staining (original magnification ×40)

arrowhead indicates brisk dermal neutrophilic infiltrate


A man in his 30s presented with reticulate hyperpigmentation, progressive since early childhood, overlying the trunk and extremities (Figure, A and B)

Physical examination revealed normal fingernails and toenails, dermatoglyphics, and dentition

Additionally, there was no evidence of alopecia or keratoderma

Review of systems was remarkable for hypohidrosis, photophobia, and a history of recurrent sinopulmonary infections, including pneumonia, requiring hospitalization

Family history was notable for similarly affected men on the patient’s mother’s side

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

A, Reticulate hyperpigmentation involving the lower and upper distal extremities, including the dorsal surface of the hand

B, Diffuse reticulate hyperpigmentation of the trunk and flexures, including the posterior neck and axilla

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

D, Congo red stain (original magnification ×200)


A woman in her early 20s presented with 1 year of discrete asymptomatic brown lesions distributed over her lower extremities

She denied any history of trauma or prior lesions at these sites and could not pinpoint any exacerbating or alleviating factors

She denied fever, arthralgias, myalgias, or constitutional symptoms

She had no significant past medical history and took no medications or supplements

A physical examination revealed several 2-mm to 4-mm round, hyperpigmented macules symmetrically distributed over the lower extremities and dorsal feet (Figure, A)

A skin biopsy was performed (Figure, B-D)


A woman in her 20s presented with numerous grouped yellow-brown and red-brown papules on the upper eyelids, infraorbital rims, bilateral axillae, inframammary creases, lower back, buttocks, hips, inguinal folds, and proximal thighs (Figure, A and B)

The lesions first appeared in her right axilla 6 months prior and were asymptomatic

The patient also noted a 3-month history of polyuria, polydipsia, and nocturia

Review of symptoms was otherwise negative

She had no notable medical or family history

On physical examination there were no other sites of involvement including mucosal surfaces

Shave biopsies were obtained from the right (Figure, C) and left axillae (Figure, D) and sent for histopathological examination

A, Clinical images show punctate yellow-brown papules on the upper eyelids and infraorbital rims

B, numerous red-brown papules in the right axilla

C, Histopathology of a shave biopsy sample from the right axilla (original magnification ×200)

D, Histopathology of a shave biopsy sample taken from the left axilla (original magnification ×200)


A young girl presented to the pediatric emergency department for evaluation of blisters on both of her hands

Three weeks prior, she had returned from school with 2 itchy papules on her right hand

The papules continued to enlarge over the course of the day and progressed to form blisters

The following day, she noticed similar lesions appearing on her left hand

She was evaluated by her pediatrician, who prescribed oral cefdinir and hydrocortisone, 2.5%, cream, but there was no substantive improvement

On further questioning, she recalled similar itchy papules appearing on her feet without progression to blisters

Her medical history was significant for atopic dermatitis that was well controlled with topical triamcinolone, 0.1%, cream

Physical examination demonstrated numerous skin-colored tense bullae on her bilateral hands (Figure 1, A)

Xerosis was on the trunk and extremities

On her dorsal and plantar feet, there were macerated, erosive plaques without vesicles or bullae (Figure 1, B)

Results of herpes simplex virus polymerase chain reaction, bacterial culture, potassium hydroxide preparation, mineral oil preparation, and direct immunofluorescence were negative

A, Bilateral palms with at least 20 tense, white-gold bullae, several with violaceous borders and overlying tapioca-like appearance, present diffusely but concentrated on the palmar surfaces

B, Bilateral dorsal feet with macerated, erosive plaques


A 60-year-old woman was referred to the emergency department with a history of abdominal discomfort and a palpable mass in the right iliac fossa

She denied any change in her bowel habits or having experienced rectal bleeding

She had a normal appetite and had no history of weight loss

Before this admission, she was healthy, with no previous medical problems

On examination, her vital signs were within normal limits

She was mildly tender in the right iliac fossa and there was a large mobile mass, at least 10 cm in diameter, that was easily palpable in the right iliac fossa

She was initially investigated with a computed tomography (CT) scan of the abdomen (Figure 1)

She underwent a colonoscopy that showed no intraluminal mucosal abnormalities or masses, but evidence of external compression at the cecum was noted

Coronal (A) and transverse (B) computed tomographic images show a large intra-abdominal cystic mass in the right iliac fossa


A man in his 70s with history of pancreatic adenocarcinoma who had undergone a Whipple procedure 2 years prior to presentation came to the emergency department with a 1-day history of waxing and waning sharp abdominal pain radiating to his back

He admitted to nausea but no vomiting, fevers, or chills

He reported having normal bowel movements and passing flatus without difficulty

On examination, he was afebrile and not tachycardic

He had a well-healed abdominal midline wound

His abdomen was soft, not distended, and mildly tender diffusely, worse in the epigastrium, without rebound, guarding, or any palpable masses

His bloodwork revealed an elevated lipase level of 2842 U/L (to convert to microkatals per liter, multiply by 0.0167) without leukocytosis or any electrolyte abnormalities

Liver function test results were within normal limits

Given a history of pancreaticoduodenectomy, the patient underwent computed tomography of the abdomen and pelvis with oral and intravenous contrast for further evaluation (Figure)

A, Axial cross-section of patient's computed tomographic scan of his abdomen and pelvis after a Whipple procedure

The stomach is distended with oral contrast and an air fluid level

A tubular solid structure can be seen within the body of the stomach

B, Coronal view demonstrating the tubular solid mass within the distended stomach


A 4-year-old white girl presented with left jaw pain and signs and symptoms of an upper respiratory tract infection

Her medical history included recurrent acute otitis media requiring tympanostomy tube placement

On physical examination, the left parotid gland was diffusely full and firm, which was consistent with viral parotitis

Gland massage, warm compress, sialagogues, and increased hydration were recommended

At the 2-week follow-up, the swelling had largely resolved with a now-firm, slightly mobile, subcentimeter mass palpable in the anterior parotid gland

Four days later the mass enlarged and became painful

A magnetic resonance imaging (MRI) study identified a 1.2 × 1.7 × 1.6-cm solid mass in the anterior parotid gland with well-defined borders and indention into the masseter muscle

The mass was isointense to muscle on T1, hyperintense on T2, with strong contrast enhancement, heterogeneous internal enhancement and a central focal area of hypointensity (Figure, A)

The patient underwent a left superficial parotidectomy

Grossly the lesion was nonencapsulated, firm, dark colored, and was located within the anterior superficial lobe of the parotid gland

Microscopically, the tumor was composed of spindle cells with elongated nuclei in a storiform pattern embedded in moderately dense admixed lymphohistiocytic infiltrate with no atypia (Figure, B)

Immunohistochemical analysis (IHC) was positive for activin-like kinase-1 (Alk-1) (Figure, C), vimentin, CD68, and factor XIIIa, and negative for smooth muscle actin (SMA), desmin, cytokeratin, and CD34.A, Postcontrast axial magnetic resonance image (MRI) demonstrating mass (arrowhead) with strong contrast enhancement, heterogeneous internal enhancement with a central focal area of hypointensity

B and C, Histopathologic images, original magnification ×200. B, Spindle cells with elongated nuclei in a storiform pattern embedded in moderately dense admixed lymphohistiocytic infiltrate with no atypia


A preadolescent girl presented with a 1-week history of left-sided facial swelling

She had presented 3 months prior with neurologic and vision changes and was diagnosed as having varicella zoster encephalitis and cytomegalovirus retinitis

Further testing led to a diagnosis of HIV/AIDS, and at that time she was started on highly active antiretroviral therapy (HAART) therapy as well as bactrim prophylaxis

Physical examination revealed left-sided facial fullness and a left-sided violaceous, vascular-appearing lesion involving the left maxillary gingiva (Figure, A)

The patient was afebrile, her white blood cell count was 3300/μL, and her CD4 count was 210 μg/mL

Magnetic resonance imaging (MRI) of the face revealed a poorly enhancing mass arising from the left maxilla and extending into the left maxillary sinus (Figure, B)

An incisional biopsy was undertaken, during which excessive bleeding was encountered

Histologic analysis and immunophenotype of the specimen showed a dual population of lymphoma cells with plasmablasts having large vesicular nuclei and centrally located eosinophilic nucleoli and “plasmacytic” cells with comparatively smaller darker nuclei and smaller scanty nucleoli (Figure, C)

Immunohistochemical expression was strongly positive for CD138 (Figure, D) and Epstein-Barr virus–encoded small nuclear RNA (EBV-EBER), and the Ki-67 index (marker of mitotic division) was greater than 90%, negative for CD45, CD20, HHV-8, TdT, CD34, PAX5, CD3, CD99, ALK-1, and CD30. Flow cytometry was noncontributory


A man in his 70s presented with a 6-year history of a painless lump in his left supraclavicular region

Palpation detected a smooth, soft, and fluctuant mass in his left supraclavicular region

Fiberscopic examination of the pharynx and larynx revealed no abnormalities

Contrast-enhanced computed tomography (CT) demonstrated a poorly circumscribed, heterogeneous, and cystic mass near the sternocleidomastoid muscle (SCM)

The SCM around the tumor was thickened, suggesting invasion of the SCM

There was no continuity between the tumor and the thyroid gland (Figure, A)

Magnetic resonance imaging revealed a poorly marginated, heterogeneous, and partially tubular mass

Fine-needle aspiration cytology revealed cystic elements but no atypical cells

Positron emission tomography showed hypermetabolism (maximum standardized uptake value, 1.6) in the mass, but no other hypermetabolic foci

At surgery, the tumor was attached to the medial side of the origin of the left SCM

No extensive adhesions were observed, and dissection of the tumor from the adjacent tissues was straightforward

The lesion measured 2.5 × 3.0 × 4.0 cm, was a solitary well-circumscribed roundish nodule with a thin capsule, and its cut surface had a yellowish white homogeneous surface (Figure, B)

Pathological examination revealed that the mass comprised an irregular combination of squamous epithelial and spindle cells, with a mature adipose tissue component (Figure, C and D)

Immunohistochemical analysis revealed that the epithelial component was diffusely positive for cytokeratin AE1/AE3.A, Contrast-enhanced neck computed tomographic (CT) image

C and D, Histopathologic images, hematoxylin-eosin

C, Original magnification ×100. D, Original magnification ×200.


A man in his 70s presented to an outside institution with a 4-month history of right-sided nasal congestion, which failed to resolve with nasal sprays, antibiotics, and oral prednisone

He also reported intermittent right-sided epistaxis occurring multiple times per day

He had not experienced prior nasal trauma or surgery and had quit smoking 30 years ago

At the outside institution, a nasal mass was found on examination and biopsied

He was referred to our office for further management

Endoscopic examination showed a dark, soft-tissue mass filling the right nasal cavity

Computed tomography (CT) of the sinuses revealed opacification of the right nasal cavity with tissue protruding into the nasopharynx and complete opacification of the right maxillary, anterior ethmoid, and frontal sinuses (Figure, A and B)

The patient underwent surgical resection

Removal of the mass required a right medial maxillectomy, septectomy, and excision of the right nasolacrimal duct via endoscopic approach

Resection also necessitated removal of the anterior maxillary sinus wall via Caldwell-Luc procedure

Histopathologic analysis from both the outside hospital and our institution revealed sheets of atypical, pleomorphic epithelioid cells, some with prominent pigment (Figure, C)

Immunohistochemical stains were positive for HMB-45 (Figure, D) and S-100.A and B, Noncontrast computed tomographic images of the paranasal sinuses


A man in his 70s was admitted to the hospital with encephalopathy, recent falls, leukocytosis, nausea, and arthralgias

On presentation to the emergency department, he received antibiotics owing to a concern for aspiration pneumonia

He underwent a stroke workup, including transesophageal echocardiography (TEE), results of which were negative

He was noted to have a progressing cutaneous eruption during his admission, and the dermatology department was consulted

He was found to have scattered petechiae and hemorrhagic vesicles, with most concentrated on the hands and feet (Figure, A and B)

Palpable purpura were noted across the extremities

Several lesions were studded with pustules

He had an enlarging bulla on his left wrist

An abrasion on his left elbow was attributed to his recent falls

The infectious disease department was also consulted, and the patient was given broad-spectrum antibiotics owing to concern about endocarditis

As part of his dermatological workup, two 4-mm punch biopsy specimens were obtained (Figure, C and D)

One was sent for histopathological examination, and the other was sent for fungal, bacterial, and mycobacterial cultures

A bacterial culture swab was obtained from the tense bulla on his left wrist

B, Acral eruption on the dorsal surface of the foot

C and D, Skin biopsy specimens, hematoxylin-eosin

C, Specimen from the dorsal side of the right foot

D, Focal ulceration and underlying vascular thrombosis with extramural fibrin deposition


Papillary/exophytic squamous cell carcinoma

A man in his 30s presented with a mass in the right nostril that had been growing for about 1 year

The lesion bled with minimal trauma but did not obstruct his breathing

The patient did not smoke and denied any medical history of chronic rhinosinusitis

Physical examination revealed a 1-cm, nontender, exophytic, verrucous, skin-colored plaque on the right nasal vestibule (Figure, A and B)

A diagnostic shave biopsy specimen was taken after informed consent was obtained (Figure, C and D)

A and B, An exophytic, verrucous, and skin-colored plaque on the right nasal vestibule

C, Histologic sections display papillary fronds with slender fibrovascular cores that are lined by nonkeratinizing squamous epithelium (original magnification ×200)

D, Higher magnification shows mucin-containing goblet cells

Neither cytologic atypia nor atypical mitotic figures are present (original magnification ×400)


A woman in her 20s developed a cutaneous eruption on her left leg 3 days after returning from a trout fishing trip in a freshwater lake

She recalled having a small abrasion on her left leg before wading waist-deep in search of trout

Prior to presentation, she was unsuccessfully treated by 3 different physicians for allergic contact dermatitis and cellulitis

Attempted treatments included topical and oral corticosteroids, trimethoprim-sulfamethoxazole, ceftriaxone, doxycycline, and clindamycin

Following 1 month of failed treatment, she presented to the dermatology clinic

Physical examination revealed erythematous, crusted, edematous plaques and pustules with weeping of yellow exudate on the left leg (Figure, A)

Plaques extended from the lower mid-shin to the thigh in a sporotrichoid spread, ending with a palpable enlarged lymph node in the left groin

The patient reported no history of cutaneous eruptions on her legs or feet, and there was no obvious thickening, yellowish discoloration, or subungual debris of the fingernails or toenails on examination

A laboratory work-up, potassium hydroxide scraping (Figure, C), and punch biopsies for histopathologic analysis and fungal, bacterial, and atypical mycobacterial tissue cultures were performed

A, Erythematous, crusted, edematous plaques and pustules on the left leg prior to definitive treatment

B, Near complete resolution of pustules and resultant postinflammatory hyperpigmentation after definitive treatment

C, Potassium hydroxide scraping taken from the left leg


Acute generalized exanthematous pustulosis

Deficiency of the interleukin-1 receptor antagonist

An 18-month-old boy with a history of atopic dermatitis presented to the emergency department with a 2-day history of high fever and rash that began 2 days after completing an 8-day course of clindamycin for sinusitis

On presentation, the child was lethargic and fussy, with a temperature of 38.1°C

Physical examination revealed a strikingly symmetrical eruption of erythematous, edematous, annular plaques with superimposed small pustules at the periphery, and associated desquamation (Figure, A and B)

The eruption was accentuated in the inguinal folds, antecubital fossae, and popliteal fossae

Laboratory evaluation was significant for leukocytosis with neutrophilia (26000 μL

to convert to × 109 per liter, multiply by 0.001)

Neutrophil levels were 66.7% (to convert to proportion of 1.0, multiply by 0.01), and lymphocyte levels were 9.9% (to convert to proportion of 1.0, multiply by 0.01)

C-reactive protein and erythrocyte sedimentation rate levels were within normal limits

Test results for anti-DNase B antibodies, a respiratory viral panel, and blood and throat cultures were all negative

A, Right arm with erythematous edematous plaque with superimposed pinpoint pustules and associated superficial desquamation

B, Bilateral buttocks with well-demarcated, erythematous plaques with pustules at the periphery

Superficial desquamation is present at the superior aspect of the buttocks

C, Hematoxylin-eosin stain (original magnification × 20)

There was no ocular or mucosal involvement and no lymphadenopathy

There was no family history of psoriasis

Biopsy of the left thigh revealed a subcorneal pustule with underlying neutrophilic spongiosis, with associated psoriasiform epidermal hyperplasia and rare individually necrotic keratinocytes

There was edema of the papillary dermis and a mild superficial perivascular lymphocytic infiltrate with scattered neutrophils (Figure, C)


Metastatic lytic bone lesions of unknown primary

A 43-year-old woman presented to the emergency department with substernal chest pain and dyspnea

After ruling out acute coronary syndrome or pulmonary embolus, a computed tomography scan of her thorax revealed lytic lesions of the sternum and second rib and a large mass involving the right scapula (Figure 1)

On further inquiry, the patient acknowledged right shoulder pain that had been progressing over 5 months and leading to a limited mobility of the right upper extremity

Cross-sectional computed tomography demonstrating a mass of the right shoulder (arrowhead)

Her comorbidities included hypertension, morbid obesity, fibromyalgia, and obstructive sleep apnea dependent on positive pressure ventilation

She underwent a tonsillectomy at age 5 years

She smoked for 1 year but quit 20 years before this presentation

There was no family history of malignancy

On examination she was morbidly obese with a body mass index (calculated as weight in kilograms divided by height in meters squared) of 63. There was fullness involving the right scapula

Laboratory testing included comprehensive metabolic panel and complete blood cell count results within the normal range, except for a calcium level of 10.4 mg/dL (for millimoles per liter, multiply by 0.25) (reference range, 8.4-10.2) and alkaline phosphatase level of 400 U/L (for micokatals per liter, multiply by 0.0167) (reference range, 36-126)

A computed tomography–guided biopsy of the mass gave a broad differential

She was referred to our Cancer Center with a diagnosis of a metastatic giant cell tumor

Magnetic resonance imaging of the shoulder was not possible because of weight limitations


A foreign body reaction (piece of glove)

A 35-year-old white head and neck surgeon with an unremarkable medical history was referred to the Surgical Emergency Department for a phlegmon of the flexor tendon sheath of the right hand

The lesion progressively developed several days after an unintentional exposure to blood with a blade used to drain an abscess of the oral cavity

The patient presented a deep wound induced by the bistoury piercing of the index finger pulp of the right hand, with a disrupted epidermal barrier

Both the patient who was operated on and the surgeon were negative for human immunodeficiency virus and hepatitis B/C virus testing

A soft tissue abscess of the digit appeared 7 days after the initial trauma

Antibiotic therapy combining amoxicillin and clavulanate was started, without any significant improvement

Surgery was performed urgently, with excision of the wound entrance and washing of the flexor tendon sheath

Bacteriological swabs were performed in the same time

An antibiotic regimen with amoxicillin/clavulanate was prescribed

Forty-eight hours after surgery, pustules appeared

Further drainage and washing of the sheath were repeated (Figure, A)

The bacteriological samples were found to be sterile

The surgeon was hospitalized for infusions combining piperacillin/tazobactam and teicoplanine, carried out over 72 hours

An oral antibiotic regimen with amoxicillin/clavulanate (3 g every 24 hours) was prescribed for 10 additional days

The lesions completely resolved within 2 weeks

One year later, the surgeon presented with prodromal burning pain of the same index finger, and a similar soft tissue abscess recurred after 48 hours at the same site of the original lesion with a small painful vesicle progressively developed (Figure, B)

A, Clinical aspect of the index finger 2 days after the second surgery

B, Recurrence of the soft tissue abscess


A man in his 30s presented with a 1-week history of worsening blurry vision and gait instability

He had been treated approximately 4 months prior at an outside facility for cerebral infarct with resultant right lower extremity weakness

On presentation, he complained of headache and nasal congestion but denied diplopia or rhinorrhea

He had no history of head trauma or surgery

Findings from the ophthalmologic examination revealed bilateral visual acuity of 20/100 with intact visual fields

Findings from a head and neck examination were otherwise within normal limits, with cranial nerves III to XII intact

Computed tomography (CT) of the sinuses with image guidance protocol revealed a 5.6 × 2.7 × 2.0-cm, well-circumscribed expansile mass occupying the left petrous apex and sphenoid sinus (Figure, A)

Magnetic resonance imaging (MRI) demonstrated that this mass was heterogeneous on T1-weighted images (Figure, B) and hyperintense on T2-weighted images

The mass did not enhance with gadolinium

The position of the internal carotid artery was not obviously displaced in either a posterior or anterior direction (Figure, A and B)

Therefore, it was not entirely clear from imaging if this lesion originated in the petrous apex or sphenoid sinus

The patient was taken to the operating room in conjunction with the neurosurgery team for an endoscopic, transnasal approach to this lesion

A wide sphenoidotomy was performed, and a cystic mass was identified within the sphenoid sinus (Figure, C)

On entering the capsule, a golden-brown fluid was encountered (Figure, D)


Extramedullary chronic lymphocytic leukemia

Langerhans cell histiocytosis of the temporal bone

A man in his 70s presented with painless left-sided otorrhea, aural fullness, and hearing loss for nearly 1 year

He had previous complaints of left-sided aural pressure and intermittent clear otorrhea that were unsuccessfully treated at another center with 2 sets of pressure equalization tubes (PETs) and 3 courses of ototopical antibiotics

In the month before presenting to us, the otorrhea had turned bloody

He had not experienced fever, malaise, weight loss, lymphadenopathy, skin lesions, or vertigo

Pertinent medical history included obesity, type 2 diabetes mellitus, allergic rhinitis, and chronic lymphocytic leukemia (CLL)

The CLL had been treated successfully with chemotherapy and had been in remission for the past 6 years

The only notable finding from the physical examination was a hypomobile left tympanic membrane with an anterior-inferior PET plugged by granulation tissue

Facial nerve function was normal bilaterally

Audiometry results demonstrated a left moderate sloping to profound mixed hearing loss and speech discrimination score of 68%

A fine-cut, temporal bone computed tomographic (CT) scan was obtained, which demonstrated near-complete opacification of the left mastoid air cells, and middle ear (Figure, A)

The patient underwent a left tympanomastoidectomy for continuing otorrhea

Bulky soft tissue was removed from the middle ear and mastoid

Cytopathologic examination revealed numerous, small, basophilic cells (Figure, B)

Immunostaining showed that the cells were CD20 positive (Figure, C) and CD3 negative (Figure, D)

A, Coronal computed tomographic (CT) scan showing near-total opacification of the left mastoid air cells

B-D, Histopathologic images, original magnification ×40. B-D, Histopathological images, original magnification ×40. B, Biopsy specimen demonstrating homogenous, small basophilic cells

C, Positive immunostaining with anti-CD20 antibodies

D, Negative Immunostaining with anti-CD3 antibodies


A woman in her 50s with a long history of allergic rhinitis was referred for progressive periorbital edema with proptosis of the left eye for the past 2 years, with associated nasal congestion, altered sense of smell, and intermittent left supraorbital pain

There was no associated history of diplopia or worsening visual acuity

She had not experienced facial trauma or sinus surgery

Treatment of allergic rhinitis with cetirizine and intranasal fluticasone had provided only partial relief

On examination, there was proptosis and periorbital edema of the left eye without any restriction of extraocular movements

The left supraorbital area was tender to palpation, with no abnormal masses appreciated

A computed tomographic (CT) scan demonstrated extensive thickening of the left frontal bone, extending over the entire roof of the orbit posteriorly (Figure, A and B)

Changes within the frontal sinus were consistent with a soft-tissue component of the mass

Subsequent magnetic resonance imaging (MRI) (Figure, C and D) showed markedly hypertrophic bone in the frontal region and a homogeneously enhancing lesion within the left frontal sinus that was hyperintense on T2-weighted images, with left extraconal superior orbital and left frontal intracranial extension with dural enhancement

A combination above-and-below approach combining unilateral frontal sinusotomy with a trephination was performed

Abnormal tissue appearing in the left frontal sinus was removed, along with diseased tissue that extended into the orbital roof and orbit

Specimens were sent for pathologic evaluation, and findings demonstrated bony spaces containing lobules and whorls of spindle cells

A and B, Preoperative computed tomography (CT)

A, Extensive thickening of the left frontal bone

B, Soft-tissue component in left frontal sinus

C and D, Magnetic resonance imaging (MRI)

D, Lesion within the left frontal sinus, with superior orbital and intracranial extension


A man in his 50s presented to the emergency department with sudden onset of left-sided neck swelling, pain, and progressive respiratory distress that started while playing basketball

He reported a 2-day history of left-sided jaw pain preceding the swelling but had no other clinically significant history

Physical examination showed trismus, rightward tracheal deviation, and a hoarse voice

The left side of his neck was diffusely swollen without palpable fluctuance, induration, or a discrete mass

A complete blood cell count revealed no abnormalities

A contrast-enhanced computed tomographic (CT) scan of the neck showed a large hyperdense mass interposed between the left common carotid artery and the hypopharynx (Figure, A-C)

There was resultant lateral deviation of the left common carotid artery and medial deviation on the supraglottic larynx and hypopharynx, resulting in airway compromise

Of note, the density of the mass was identical to that of the adjacent vasculature

There was extensive edema surrounding the primary abnormality

Additional findings include periapical dental abscesses in 2 teeth (Figure, D)


Colonic ileus/colonic pseudoobstruction (Ogilvie syndrome)

A 77-year-old institutionalized and bedridden man with severe Alzheimer dementia and long-standing schizophrenia was admitted to the emergency department for bowel obstruction with progressive abdominal distension and tenderness

His family members and caregivers accompanied him

At physical examination the patient was pale, sweaty, peripherally hypoperfused, tachypnoeic, and dehydrated, and the abdomen was extremely distended, diffusely tympanic, and tender at palpation

His blood pressure was 90/70 mm Hg, his heart rate was 114 beats per minute, his oxygen saturation was at 90%, his arterial blood gas pH level was 7.43, his partial pressure of carbon dioxide was 32, his partial pressure of oxygen 68, his laceration lactate level was 5.0, and his white blood cell count was 24 000 mm3. A plain abdominal radiograph showed a huge colonic distension (Figure 1A)

A computed tomography scan with multiplanar reconstruction was also performed (Figure 1B)

A, A plain abdominal x-ray shows a huge colonic distension and a clear coffee-bean-like or “bent inner tube” sign is visible

B, A computed tomography scan with multiplanar reconstruction


Small-bowel intussusception secondary to pelvic adhesions

A 45-year-old woman presented to the emergency department on the morning after Thanksgiving with sudden, severe, constant epigastric pain that migrated to the right upper quadrant as well as subjective fever and emesis since the previous evening

She reported a failure to pass stool or flatus since the onset of symptoms

She denied hematochezia, melena, or hematemesis

Her medical history was significant for heavy smoking, reflux, and surgical procedures including a right inguinal hernia repair, total abdominal hysterectomy, and bilateral salpingo-oopherectomy, all more than 5 years prior

On examination, she had a distended and tympanic abdomen with tenderness, guarding, and rebound in the epigastrium and right upper quadrant

Vital signs and standard laboratory test results were unremarkable

An upright abdominal plain radiograph showed a distended, thickened stomach and multiple dilated loops of small bowel with air-fluid levels

Contrast-enhanced computed tomography demonstrated a focal area of mixed fat density involving the distal small bowel concerning for a lead point with adjacent mesenteric fat stranding (Figure 1)

The scan also revealed multiple bilateral lung nodules

A, Coronal view demonstrating obstructing intraluminal lesion (arrowhead)

B, Transverse view demonstrating obstructing intraluminal lesion (arrowhead)

The patient was subsequently taken to the operating room for exploratory laparotomy and found to have an obstruction in the distal ileum


Low-grade malignant peripheral nerve sheath tumor

A man in his 50s was referred for a 6-month history of swelling involving the nasal bridge and right eye, accompanied by intermittent ipsilateral epistaxis

His medical history was notable for allergic rhinitis and well-controlled type 2 diabetes mellitus

The prior biopsy diagnosis from the area of swelling was inflammatory nasal polyp

Physical examination revealed tenderness to palpation over the right nasal bridge and mild right proptosis

Flexible and rigid nasal endoscopy demonstrated a right-sided exophytic mass visible between the middle turbinate and nasal septum

Computed tomography showed a large, right-sided mass centered in the ethmoid sinus with expansion into the anterior skull base, anterior cribriform plate, and right medial orbit

Magnetic resonance imaging showed vascularity in the lesion and early intracranial involvement with transdural spread but no evidence of invasion into the meninges or brain

The patient was taken for surgery, and findings from intraoperative frozen section analysis were consistent with a spindle cell neoplasm

Definitive skull base resection of the mass was deferred until final pathological diagnosis was obtained, given the morbidity with skull base resection

The final pathology report showed an infiltrative, low-grade, malignant spindle cell neoplasm with immunoreactivity for BCL2 protein and neural marker, S-100 protein, and focal immunoreactivity for smooth muscle markers SMA and HHF35 (Figure)

Cytoplasmic immunoreactivity for β-catenin was noted, and nonreactive immunostains included CD31, CD34, epithelial membrane antigen (EMA), and CD99. In addition, there was minimal mitotic activity, and no tumoral necrosis was present

There was no regional or distant metastasis, and TNM staging was cT4N0M0.Histopathologic photomicrographs of a biopsy specimen from a mass centered in the ethmoid sinus

B, Invagination of respiratory-type epithelium

C, Tissue section showing strong nuclear and cytoplasmic immunologic staining for S-100 protein

D, Immunologic staining for smooth muscle actin showing patchy immunoreactivity in tumor cells


A woman in her 60s presented with mild to moderate trismus and swelling on the left side of the face of a few months’ duration

Her medical history was significant for herpes zoster and self-limited vertigo approximately 3 years prior

Her physical examination was remarkable for left-sided facial swelling

No deficits of the facial or trigeminal nerves were detected

Magnetic resonance imaging (MRI) was performed (Figure)

T1-weighted image demonstrating a mass lying on the anterior surface of the eroded left mandibular condyle with homogeneous isointensity relative to skeletal muscle

B, Postcontrast T1-weighted image with fat suppression showing homogeneous, avid enhancement of the mass

C, T2-weighted image showing homogeneous moderately hyperintense signal

D, Diffusion-weighted image is significant for marked diffusion restriction


HIV-associated benign lymphoepithelial cyst

A white man in his 40s presented with a slowly enlarging, nontender, left-sided neck mass at the angle of the mandible that had been present for 4 years

Fine-needle aspiration was performed twice, each time with inconclusive results

Computed tomography (CT) of the neck showed a heterogeneous mass located immediately adjacent to the tail of the left parotid

He underwent a left superficial parotidectomy with en bloc removal of the mass

Pathologic findings revealed a 3.0 × 2.9 × 1.9-cm, encapsulated, tan, soft nodule within the specimen

The nodule had peripheral cystic spaces as well as focal areas of hemorrhage and a 0.5 × 0.4 × 0.4-cm area of calcification within the periphery

The entire tumor was confined within a periparotid lymph node, almost completely replacing it

Histopathologic findings (Figure) showed a mixture of dark, granular, basophilic acinar cells and eosinophilic intercalated ductal cells

The acinar cells contained periodic acid–Schiff–positive, diastase-resistant intracytoplasmic granules, whereas the ductal cells were both fusiform and cuboidal forming small ductlike structures

The neoplasm also exhibited focal areas of hyaline sclerosis without necrosis


A teenage boy was referred with a progressive 2-week history of blurred vision in his right eye and right ear pain, which had worsened in the past 7 days

This change prompted a visit to an optometrist, who found a mild decrease in visual acuity and minor cataract in the right eye

His primary care physician diagnosed his ear pain as acute otitis media and prescribed a course of amoxicillin-clavulanate

however, the young man’s vision continued to deteriorate and became associated with the onset of right eye swelling and forehead tenderness

A repeat eye examination by an ophthalmologist was performed and showed normal peripheral visual acuity with a decrease in central vision and confirmed proptosis

The patient had not experienced nasal congestion, epistaxis, weight loss, fevers, or chills

Physical examination included right eye proptosis and restricted eye movement

A computed tomographic (CT) scan with contrast of the face showed a small relatively homogenous mass in the right anterior nasal cavity invading the right orbit, and a magnetic resonance image (MRI) with contrast showed a right superior nasal cavity mass, with invasion of the right orbit and right ethmoidal sinus with extension to the overlying soft-tissue scalp (Figure)

A 30° endoscope was used to visualize the superior nasal cavity and revealed a fleshy red soft-tissue mass

Several biopsy specimens were taken and sent for frozen pathologic evaluation

A, Postintravenous contrast computed tomographic (CT) scan of the face showing a relatively homogenous mass of the right ethmoid complex with invasion of the orbit and extension to the nasal cavity (arrowhead)

B, Fast spin echo (FSE) T2 magnetic resonance image (MRI) of the right ethmoidal/orbital soft-tissue mass (arrowhead)

C, Positron emission tomographic (PET) scan of the face with fludeoxyglucose F 18 showing increased uptake within the right ethmoid air cells and nasal cavity, and in the medial right orbit


A woman in her 30s noticed a painless left neck mass

She had no history of dysphagia, dyspnea, recent upper respiratory tract infection, injury, or surgery

She also had no weight loss, pain, or fever and had no history of tobacco or alcohol use, radiation exposure, family history of thyroid cancer, and no previous thyroid dysfunction

Thyroid function test results were normal

Physical examination revealed a palpable nontender, firm mass and no cervical lymphadenopathy

She had a medical history of high-grade osteosarcoma with areas of leimyosarcoma of the tibia which had been treated with right above-knee amputation and chemotherapy 4 years prior to presentation

She was followed up in the oncology clinic without any recurrence or metastases

She was also diagnosed with squamous cell carcinoma of the cervix 2 months earlier and was awaiting surgery

Computed tomography (CT) of the neck revealed a 6.0 × 3.4 × 4.4-cm calcified left thyroid lobe mass (Figure, A)

Positron emission tomography–computed tomographic (PET-CT) imaging showed increased uptake in the left thyroid gland

The neoplastic cells were positive for p53, vimentin, and smooth muscle actin, and negative for S-100, thyroid transcription factor-1 (TTF-1), and CD34 (Figure, B)

A, Computed tomographic image reveals a calcified mass (blue arrowhead)

Left, negative immunostain results for S-100

right, positive immunostain results for vimentin (original magnification ×100)


A white woman in her 60s presented for a routine visit to the outpatient dermatological graft-vs-host disease (GVHD) clinic at Westmead Hospital for review of lichenoid GVHD evolving into sclerodermatous GVHD

The GVHD occurred secondary to a bone marrow transplant in 2014 for treatment of chronic lymphocytic leukemia

At the time of review she was being treated with oral azathioprine (50 mg twice daily), tacrolimus (0.5 mg twice daily), and prednisone (25 mg daily) therapy

She was also receiving monthly intravenous immunoglobulin, prophylactic oral fluconazole, acyclovir, sulfamethoxazole-trimethoprim, and penicillin

One month prior to commencing oral azathioprine therapy, the patient noted the growth of nodules on the genital and perianal region, with 1 ulcerated nodule in the perianal area associated with painless bleeding on trauma

On examination she had several yellow-colored nodules, some of them ulcerated with extrusion of yellow-colored discharge on the left labia majora and on the perianal area with surrounding inflammatory reaction (Figure 1)

Additionally, she had sclerodermatous plaques on the lower legs, scarring alopecia on the scalp with minimal hyperkeratosis, nail dystrophy and lichenoid changes, erythematous changes in both feet, and desquamation in the lower legs and on the back and arms suggestive of eczematous or postlichenoid changes as part of her lichenoid GVHD

Initial investigations revealed a white blood cell count of 13.1 × 103/μL (to convert to billions per liter, multiply by 0.001)

however, the results of the rest of her blood tests including the full blood count and electrolytes, urea and creatinine, and liver function tests were otherwise stable and unremarkable

Clinical photographs of cutaneous nodules in a woman with graft-vs-host disease

A, Multiple erythematous eroded nodules in the perianal area

B, yellowish nodules located on the left labia majora


A black woman in her 50s presented to the clinic with a 3-month history of nontender mildly pruritic verrucous nodules in several areas of a polychromatic tattoo on her right lower leg and foot

She reported that these appeared 1 to 2 months following placement of the tattoo and were restricted to areas where the artist created pink zones by layering white ink over red ink

She denied pain, fevers, and chills and had no history of skin cancer

She reported a distant history of discoid lupus, and was taking 2 antihypertensives but no other oral or topical medications

Physical examination revealed well-demarcated erythematous to violaceous verrucous nodules on the right lateral leg and dorsal foot (Figure 1A)

A punch biopsy specimen from a representative nodule was obtained, with an additional biopsy for tissue culture (Figure 1B)

A, Verrucous nodules are restricted to zones of pink pigment within the tattoo, sparing other colors present

B, Histologic reaction pattern from punch biopsy specimen of right lower leg nodule (hematoxylin-eosin, original magnification ×4)


A 22-year-old woman with a history of systemic lupus erythematosus was readmitted with fever and a generalized eruption

Six days prior to this admission, she had been hospitalized for a photo-induced flare of acute cutaneous lupus erythematosus (ACLE)

At readmission, she was febrile with a temperature of 101.5°F, and on examination, she had a worsening, exquisitely tender rash with several blisters across her right arm

Physical examination revealed erythematous plaques with superficial desquamation in the malar distribution of the face, nose, ears, and medial forehead (Figure, A), as well as bilateral extensor arms, chest, posterior neck, and upper back

Several large bullae were located on the right arm (Figure, B)

Numerous flat and raised 2-zoned atypical targets with dusky centers were appreciated on the forearms, abdomen, back (Figure, C), thighs, legs, palms and soles

A, Dusky malar erythema with focal heme crusting

C, Diffuse typical and atypical target lesions of the back

D, High-power view (original magnification 100×) of punch biopsy obtained from left abdomen demonstrating dyskeratosis with superficial perivascular inflammation and interface changes

Hemogram test results revealed a normocytic anemia (mean corpuscular volume, 99 fL

hemoglobin, 11.7 g/dL [to convert to g/L, multiply by 10.0]

hematocrit, 33.4%), in addition to thrombocytopenia (platelet count, 81 K/μL)

Other pertinent laboratories included hypocomplementemia (C3 of 42 mg/dL

C4 of 3 mg/dL), a speckled antinuclear antibody titer of 1:1280, and an antidouble-stranded DNA titer of 1:80, along with positive anti-Smith, antiribonucleoprotein, and SSA/anti-Ro antibodies

Bacterial blood, urine, stool and cerebrospinal fluid cultures, and a hepatitis panel revealed no infectious source

Punch biopsy of an abdominal atypical target lesion was performed (Figure, D)


Demyelinating disease (multiple sclerosis or acute disseminated encephalomyelitis)

A 21-year-old man was admitted to the hospital owing to recurrent, transient episodes of dysarthria, dysphagia, and limb weakness over the course of 3 days

Although these symptoms had resolved after 3 hours’ rest, they recurred 2 hours prior to hospital admission

In addition, the patient had begun to experience weakness and numbness of his limbs, with more severe symptoms on the right side

The symptoms began to gradually resolve 2 hours following admission, and normal function had returned within 2 days

No significant history of illness was noted in the patient or among family members

Neurological examination on admission revealed normal mental status, and the results of cranial nerve examination were normal

Although the patient had cavus feet, no atrophy was observed in the distal lower limbs (Figure, A)

Muscle strength was classified as grade 4 for the left limbs and grade 3 for the right limbs, according to Medical Research Council criteria, and muscle tone was diminished

The sensory examination was unremarkable

However, bilateral positive Babinski signs were observed

Results from laboratory examinations, including blood electrolytes, cerebrospinal fluid, and lactate, were normal

Oligonucleotide bands and aquaporin 4 detection were negative

Craniocervical computed tomographic angiography results were normal

Cerebral magnetic resonance imaging (MRI) revealed abnormal bilateral increases in T2 signal and diffusion restriction in the periventricular areas and the splenium of corpus callosum (Figure, B)

Interestingly, a significant reduction of the abnormal signal was noted on MRI 3 months later

Electrophysiological examination of peripheral nerves revealed extensive bilateral nerve conduction abnormalities

Significantly reduced motor nerve conduction velocity was also observed in the median nerves (38.3 m per second in the left and 36.5 m per second in the right)

B, Patient’s brain axial diffusion-weighted image at admission


A 66-year-old woman with a remote history of a pituitary macroadenoma was referred to the neuro-ophthalmology clinic for intermittent diplopia of several years’ duration

Diplopia was frequently present after repeatedly changing direction of gaze

There was no associated headache or ocular or periorbital pain

She had not noticed any injection of the sclera

Her family and friends made no comments regarding exophthalmos

She had not noticed any deficits in axial or appendicular strength, rapid fatigability, or dysphagia

Her symptoms did not exhibit diurnal variation

She had not noted any changes in her hair, skin, or nails

She had no subjective disturbance of thermoregulation

Her relevant history included a pituitary macroadenoma, which had been resected 30 years prior, followed by radiation

There was no evidence of subsequent recurrence

There was no family history of ptosis, ophthalmoplegia, or myopathy

The patient was a retired English teacher

Examination demonstrated anisocoria (right pupil larger than left), which was accentuated in bright ambient light

and mildly restricted supraduction, adduction, and infraduction of the right eye (Video, prepared by N

The second half of the Video (from 24 seconds on) demonstrates marked restriction of elevation, depression, and abduction in the right eye after eccentric gaze

The patient’s neurological examination demonstrated a normal level of consciousness, fluent speech, normal comprehension, good attention, normal cranial nerve function aside from the aforementioned deficits, full strength, normal sensation to proprioception and temperature, symmetric reflexes, absence of ataxia, and a stable, narrow-based gait

A recent contrast-enhanced brain magnetic resonance image showed a stable intracanalicular vestibular schwannoma on the left, which had been monitored for 8 years


Fragile X–associated tremor ataxia syndrome

A 58-year-old man presented with a 1-year history of severe and progressive action tremor in his dominant hand when writing

When questioned, he described subtle balance problems without falls developing over the past 3 years

With the exception of profound difficulties with handwriting, motor symptoms did not affect livelihood or activities of daily living

There was no tremor reported in the nondominant hand or other limbs or symptoms of autonomic dysfunction

Neither he nor his wife reported any changes in cognitive function

His medical history comprised surgeries of the hand (laceration repair), leg (fracture repair), knee (reconstruction), and ear (stapedectomy)

these were not related to the presenting symptoms

He had a family history of ischemic heart disease (both parents) but no family history of neurological disorder

He exercised regularly (5 times per week) and consumed 2 to 4 standard drinks per week

He was a former smoker with a 2-year pack history and denied any illicit drug use

Cognitive testing revealed deficits in executive function and speed of information processing

Examination of eye movements and speech revealed jerky eye pursuit, saccadic dysmetria, and moderate slurring of speech

Additional motor signs included abnormal handwriting (Figure 1A), bilateral intention tremor, mild rigidity (upper extremities), and moderately impaired heel tapping

He demonstrated moderate body sway while standing, was unable to stand on 1 foot for more than 10 seconds, and had impaired tandem walking

Gait, posture, and walking capacities during a timed 6-m walk were normal

A, Handwriting task from the motor examination showing abnormal handwriting

B, T2-weighted fluid-attenuated inversion recovery image from brain magnetic resonance imaging showing white matter hyperintensities in the middle cerebellar peduncles

Brain magnetic resonance imaging showed moderate volume loss in the cerebral hemispheres bilaterally as well as the cerebellum and brainstem

Abnormal signal intensity was noted in the cerebral hemispheric white matter bilaterally, the middle cerebellar peduncles (Figure 1B), the cerebellar white matter, and the pons


An 11-day-old, full-term girl presented for evaluation of multiple skin changes that were noted at birth (Figure)

These skin changes included a red patch on the central lower back with a soft nodule located on top of the patch

Additionally, she had a pedunculated red papule positioned on top of the red stain and soft nodule

She was asymptomatic, moving all limbs, urinating, and defecating normally

Her prenatal screens, including 20-week ultrasonography, were normal

Pregnancy was uncomplicated and there was no history of teratogenic exposure or family history of congenital defects

Ultrasonography of the spine obtained prior to the visit showed no abnormalities

Photograph depicting a red patch with overlying soft nodule and red pedunculated papule in the lower midback of a neonate


An elderly woman presented with 2 episodes of vertigo lasting a few minutes while stationary over 1 day and a several-year history of right-sided hearing loss

She had not experienced otalgia, otorrhea, or tinnitus

Physical examination revealed a mass obstructing the right external auditory canal and House-Brackmann grade 2 facial nerve weakness on the right

Audiogram revealed right-sided moderate to profound mixed hearing loss

Computed tomography revealed a soft-tissue mass emanating from the middle ear involving the facial canal with surrounding bony erosion, including dehiscence of the tegmen, cochlea, bony labyrinth, lateral semicircular canal, and jugular foramen

The patient underwent transtemporal mastoidectomy and debulking

However, owing to the mass’s vascularity and significant bleeding, the operation was aborted, and she was sent for embolization (Figure, A)

Magnetic resonance imaging during embolization revealed intense enhancement in the right middle ear extending from the epitympanum to the hypotympanum with extension into the right external auditory canal (Figure, B)

She returned to the operating room, and the vascular mass was found to extend inferiorly to the jugular bulb and carotid and anteriorly into the eustachian tube

The mass was adherent to the underlying bone and had invaded the facial canal but not the nerve itself

Immunohistochemical stains of the specimen indicated cells positive for synaptophysin with a sustentacular pattern of cells positive for S-100 (Figure, C and D)

A, Mastoidectomy, visualization of vascular mass

B, T1-weighted axial magnetic resonance image (MRI) showing enhancement of right middle ear extending to external auditory canal

C, Hematoxylin-eosin, original magnification ×40. Clusters of cells surrounded by vascular tissue

D, Synaptophysin stain, original magnification ×40. Nests of strongly staining neuroendocrine cells separated by vasculature