Leukocytoclastic vasculitis from COVID-19 vaccine

A 71-year-old man presented to the dermatologist with a 6-week history of a rapidly progressing rash and swelling of the left side of the chest wall

He had noticed the rash a few days after receiving the second dose of an mRNA COVID-19 vaccine (Moderna)

He denied itching, weeping, bleeding, or discharge from the rash or the nipple

He had a history of metabolic syndrome treated with antihypertensives and oral hypoglycemics

The physical examination showed a morbidly obese White man

The skin examination showed a 19 × 10-cm pink, fallacious purple color deep-infiltrated papule in the left breast and a similar 15 × 7-cm papule under the left clavicle (Figure, A)

The breast examination was notable for bilateral gynecomastia and inverted left nipple without any palpable lumps

His laboratory results showed stage 2 chronic kidney disease

Results of a viral panel including hepatitis A, B, and C and HIV were negative

The punch biopsy of the skin showed a diffuse island of atypical cells in the dermis (Figure, B)

On immunohistochemical staining, the atypical cells stained positive for CK-7, GATA-3, and PAX-8 (weak focal staining) and negative for estrogen receptor (ER)/progesterone receptor (<1%), TTF-1, SATB-2, CDX-2, and NKX-3.1. The positron emission tomography–computed tomography scan showed a diffuse, infiltrating fluorodeoxyglucose (FDG)-avid cutaneous lesion on the left chest wall (standardized uptake value, 6.5) and multiple FDG-avid nodes in the axilla (bilaterally), left internal mammary, and left cervical chain

The core biopsy from the lymph node is shown in the Figure, C

B and C, Hematoxylin-eosin stains of the punch biopsy of the skin and core biopsy of the lymph node, respectively (both original magnification ×10)


A 55-year-old male jazz musician with a medical history that was significant for hypertension, coronary artery disease, and obstructive sleep apnea presented with an enlarging right more than left-sided neck mass of more than 2 years’ duration

The patient was seen and evaluated by a local surgical oncologist with a working diagnosis of possible lymphoma

The growing neck mass was causing solid food dysphagia and difficulty breathing at night

He did not have any neurological deficits

On further questioning, he had a similar mass that was treated with liposuction 10 years prior

His family history was negative for any head and neck benign or malignant disease

His social history was negative for tobacco or alcohol use

This patient was not taking any steroid medications

Physical examination showed an enlarged right more than left-sided neck mass

It was tense but compressible to palpation

Flexible fiber-optic laryngoscopy showed a crowded oropharynx with lingual tonsil hypertrophy and narrow oropharynx and hypopharynx

An outside computed tomography scan showed diffuse symmetric fatty infiltration of the pharynx, larynx, trachea, and esophagus

A magnetic resonance imaging scan with and without contrast (Figure, A) showed extensive areas of fat deposition within the neck that bilaterally extended from the level of C1 in the retropharyngeal space to the level of the sternal notch

Given that the patient was symptomatic, surgical intervention was recommended

Preoperative magnetic resonance imaging (A) and gross specimen (B)

Surgery was approached like a neck dissection

The entire specimen was removed, including its capsule, which measured 17.0 × 14.0 × 5.0 cm (Figure, B)

Six months later, a postsurgical magnetic resonance imaging scan showed that the fat prominence had largely been resected


Inflammatory linear verrucous epidermal nevus

Porokeratotic eccrine ostial and dermal duct nevus

A school-aged boy presented with occasionally pruritic, generalized hyperkeratotic papules with central white spicules and comedo-like plugs that were in a linear distribution on his face, trunk, and extremities since birth

Some of the eruptions on his trunk had spontaneously resolved with developmental growth

There was nonconsanguineous marriage of his parents, and no family members had similar lesions

The patient was otherwise in good general health and without developmental delay

Physical examination showed generalized, linear hyperkeratotic papules with numerous central small filiform keratotic white spicules and brown plugs on the head, face, neck, trunk, and bilateral upper and lower extremities (Figure, A and B), including his palms and soles along Blaschko lines, accompanied with alopecia of the scalp and eyebrows

The examination of nails, mucosae, and teeth was unremarkable

No extracutaneous involvement was observed

Dermoscopic examination showed many central white spines on the face (Figure, C) and brown cores surrounded by yellow crater-like rings on the right palm

A skin biopsy specimen was taken from the hyperkeratotic papules with white spicules on his right thigh (Figure, D)

A, Generalized, linear hyperkeratotic papules and plaques with numerous white spicules and plugs on the head, face, neck, and both arms are accompanied by alopecia of the scalp and eyebrows

B, Generalized hyperkeratotic papules with numerous central small filiform keratotic white spicules on the face (close-up view)

C, Dermoscopic examination on the face showing many central whitish spines

D, Skin biopsy specimen taken from the right thigh showing hyperkeratosis, acanthosis, and parakeratotic columns overlying the dilated epidermal invaginations, as well as thinner granular layers and dilated eccrine ostia and acrosyringia under the parakeratotic columns (hematoxylin-eosin)


A female patient in her late 40s presented with a 3-year history of nonhealing ulcers in her groin and axillae

These lesions started as itchy, weepy, reddish papules and plaques, which gradually progressed to form ulcers and were associated with pain and pus discharge

They did not heal completely after multiple courses of topical as well as systemic antibiotics, antifungals, and steroids

She frequently experienced scaling and greasiness on her scalp, which was treated with shampoos and topical steroids

She also complained of polyuria and polydipsia for the past 4 years

General examination revealed multiple mobile, firm, nonmatted, and nontender lymph nodes in cervical and inguinal region

On systemic examination, hepatomegaly (2 cm below the right costal margin) was seen

There was also bilateral enlargement of the parotid area, which was tender on palpation

Cutaneous examination revealed yellowish greasy scales on the scalp, suggestive of seborrheic dermatitis

Erythematous edematous papules were present over the retroauricular areas and the inframammary folds (Figure, A)

On examination of external genitalia, deep ulcers were observed in the bilateral inguinal folds, resembling knife-cut ulcers (Figure, B)

Similar lesions were present over the junction of labia majora and labia minora and were associated with yellowish pus discharge

A 3.5-mm punch biopsy specimen was obtained for histopathological examination from the edge of an ulcer on one of the groin folds (Figure, C and D)

A, Erythematous and edematous papules in the inframammary area

B, Deep ulcers in the groin folds resembling knife-cut ulcers with yellowish pus discharge

C, Histopathological examination of the area with intact epidermis shows dense infiltration of the ulcer bed by atypical cells

These cells were large and had characteristic grooved vesicular nuclei and moderate amphophilic cytoplasm

D, Immunohistochemistry shows diffuse positivity for CD1a in the lower epidermis and dermis


Late-onset Pompe disease (glycogen-storage disease type 2)

Sporadic late-onset nemaline rod myopathy

A 72-year-old man presented with mild proximal weakness developing into progressive camptocormia, head drop, and prominent dysphagia over a 2-year period

He reported a 20-kg weight loss and numbness to the ankles

His medical history was significant for thyroid cancer treated with total thyroidectomy resulting in a chronic left accessory nerve injury, type 2 diabetes, hypertension, and gout

His medications were metformin, allopurinol, perindopril, thyroxine, and rabeprazole

He had no history of smoking and drank 3 glasses of wine per week

Examination revealed significant wasting of proximal and paraspinal muscles with a flexed spine posture in the upright position (Figure 1A)

He had symmetric Medical Research Council grade 4/5 weakness of neck flexion, shoulder abduction, elbow flexion and extension, and hip flexion and extension bilaterally

He had weakness of left shoulder elevation and winging of the left scapula secondary to the accessory nerve injury

His deep tendon reflexes were normal and symmetric

Pinprick sensation was reduced in both feet

Lateral profile of the patient (A) revealing a flexed spine posture (camptocormia) with marked generalized muscle atrophy

B, A Gömöri trichrome stain of frozen left vastus muscle (original magnification ×400) reveals 3 atrophic muscle fibers with a patchy granular appearance (red arrowheads)

Metabolic, endocrine, autoimmune, and infectious screens had unremarkable findings

A myositis panel demonstrated low-positive Mi2 antibodies

Serum electrophoresis and immunofixation identified a small immunoglobulin G (IgG) κ paraprotein with normal findings on bone marrow biopsy

A whole-body positron emission tomography scan was normal

Nerve conduction studies revealed a mild axonal sensorimotor polyneuropathy

Concentric needle electromyography showed no spontaneous activity with widespread small, brief, polyphasic units, particularly in proximal and axial muscles, consistent with a myopathic process

An initial deltoid muscle biopsy did not show evidence of myopathy

Because the patient’s symptoms were worsening, a second muscle biopsy of vastus lateralis was performed

This revealed atrophic myofibers with a patchy granular appearance (Figure 1B)


A previously healthy 12-year-old boy presented with a 3-month history of nasal obstruction, progressive dysphonia, recurrent deep neck abscesses, and tender, bulky cervical lymphadenopathy

Computed tomography (CT) of the neck with contrast at his initial hospital admission was concerning for suppurative lymphadenitis

Despite incision and drainage, and intravenous ampicillin and sulbactam, his cervical lymphadenopathy persisted

Results of subsequent workup—including Haemophilus, tetanus, and pneumococcal antibody titers

and antinuclear antibody level—were within normal limits

Magnetic resonance imaging of the neck with gadolinium contrast demonstrated bilateral, bulky, enlarged cervical lymph nodes, as well as enhancing nodules in the left laryngeal ventricle (2.3 × 1.6 × 1.2 cm), right false vocal cord, and right tracheal wall (0.6 × 0.4 cm)

Findings from nasal endoscopy, awake flexible laryngoscopy, and operative microlaryngoscopy revealed yellow submucosal masses in the left nasal cavity, left laryngeal ventricle, and right trachea, as well as left vocal cord paresis (Figure, A)

Biopsies were taken of the tracheal, laryngeal, and nasal cavity masses, and an excisional biopsy was taken of a left cervical lymph node (Figure, B)

Histopathologic examination revealed a prominent infiltrate of benign-appearing histiocytes with numerous forms showing emperipolesis and some nuclei showing distinct central nucleoli

Immunohistochemical staining in these cells was positive for S100 and negative for CD1a

A, Awake flexible laryngoscopy showing a yellow mucosal mass in the left laryngeal ventricle

B, Hematoxylin-eosin–stained excisional lymph-node specimen at ×1000 magnification revealing histiocytes with central nucleoli and cytoplasm containing intact lymphocytes (emperipolesis)


Multifocal squamous cell carcinoma in situ

A 19-year-old male presented for evaluation of painful lesions on the tongue that had been present for at least 3 months

He stated that the pain was exacerbated by spicy foods

He had initially presented to a primary care practitioner, was prescribed clotrimazole lozenges, and reported mild initial improvement followed by symptom progression

Subsequently, the practitioner referred the patient to an otolaryngologist

The patient denied any enlarged lymph nodes, fevers, fatigue, rash, weight loss, or abnormal gastrointestinal symptoms and had no noteworthy medical history

He reported mild alcohol consumption, denied any cigarette or illicit substance use, and endorsed engaging in oral and anal intercourse

A physical examination was notable for bilateral raised leukoplakia on the tongue

it was larger on the right side than the left and had a slightly hairy appearance

The lesion was tender to touch and could not be scraped off (Figure 1A)

The lips and oral mucosa were without lesions, masses, or ulcers

The patient had no cervical lymphadenopathy

The findings of the physical examination were otherwise unremarkable

A, Clinical image of the right anterolateral tongue showing a 1 × 3 cm plaque over tongue mucosa

Left lateral tongue (not shown) had multiple gray papular lesions with similar appearance

B, Hematoxylin-eosin stain of right tongue biopsy specimen revealing prominent lymphoplasmacytic infiltrate involving squamous mucosa

no cellular atypia is seen among lymphocytes

A biopsy procedure of the right oral tongue lesion was performed, and hematoxylin-eosin staining of the specimen revealed dense perivascular plasmacytic infiltration and neutrophilic inflammation (Figure 1A)


A school-aged girl presented to the dermatology clinic with a 6-year history of hair loss and short hair

Her scalp hair was relatively normal in terms of density and length at birth

Since the age of 1 year, the girl had begun to have frequent hair loss without pulling

Her mother also found that her hair grew to approximately the same maximum length down to the upper neck without trimming

On clinical examination, diffuse, short, sparse, lanugolike, black hair over the scalp was found (Figure, A and B)

The surface of the scalp was smooth without inflammatory changes or focal alopecic patches

No involvement of eyebrows, eyelashes, nails, teeth, or body hair was found

Her intellectual performance was normal at presentation and no developmental delay had ever been noted

She denied experiencing a stressful event recently, and no habitual pulling of hair was observed by her family

Family history regarding hair loss was unremarkable

A and B, Diffuse, sparse, and abnormally short hair over the entire scalp is shown

C, Punch biopsy showed a mild lymphocytic perifollicular infiltrate and increased telogen hair with decreased anagen-to-telogen ratio (hematoxylin-eosin staining, magnification 2x)

The hair pull test was positive with predominately anagen hair extracted

Light microscopy of scalp hair shafts revealed no structural abnormalities, such as cuticular damage, twisting, bending, or miniaturization

Blood levels of ferritin, zinc, and hemoglobin were normal

A punch biopsy from the alopecic area of the scalp was performed (Figure, C)


A woman in her 50s presented with a 3-month history of rapidly spreading intensively pruritic papules on her extremities

The lesions consisted of scattered, erythematous, annular papules measuring up to 5 mm wide (Figure 1)

Further examination revealed a few asymptomatic brown flat papules scattered on her face

the palms, soles, and oral mucosa were not involved

The patient had no remarkable medical or family history and was not concurrently being treated with any medications

She had been previously diagnosed with eczema and treated with topical steroid ointments and oral compound glycyrrhizin tablets, which had no obvious effect

Clinical images of keratotic papules diffusely distributed on an extremity, original (A) and magnified (B)

Laboratory investigations, including routine blood, liver and kidney function, antistreptolysin O, C-reactive protein, antinuclear antibody, and rheumatoid factor tests, disclosed no abnormal findings

A biopsy specimen was obtained for histopathologic examination


Fragile X–associated tremor/ataxia syndrome

A 55-year-old female patient with a history of hypercholesterolemia and anxiety was referred for progressive imbalance while walking and intense fear of falling for the past 14 years

The patient had become progressively disabled and had been using a wheelchair for 4 years prior to presentation

Neurological examination showed gaze-evoked horizontal nystagmus in both directions along with slow saccades and gaze restriction in both vertical and horizontal planes

Tone was normal in all 4 limbs but stretch reflexes were absent and plantar fascia were mute

Ataxia was seen on finger-to-nose and heel-knee-shin tests

Ankle inversion with bilateral hammertoes was noted

Electromyography revealed sensory neuronopathy

On brain magnetic resonance imaging (MRI), diffuse cerebellar atrophy was noted along with T2 hyperintensities in bilateral middle cerebellar peduncles, cerebellar white matter, pons, and bilateral thalamus (Figure)

Psychiatric treatment was commenced for her severe anxiety but did not relieve the fear of falling

Magnetic resonance brain images showing T2 fluid-attenuated inversion recovery hyperintensities


Idiopathic bilateral vocal fold paralysis

Delayed iatrogenic bilateral vocal fold paralysis

A 68-year-old man with a medical history of coronary artery disease, atrial fibrillation, and obesity had undergone C5 anterior corpectomy and fusion (C4-6) with expandable cage, autograft, and anterior instrumentation and posterior spinal decompression and fusion (C4-7) for cervical spondylotic myelopathy

Five months after surgery, he presented to the otolaryngology clinic with 3 weeks of mild odynophagia and dysphagia

It was noted that the patient had a prominent posterior pharyngeal wall, subtle asymmetry of vocal fold motion, and pooling of secretions in the left pyriform (Video 1)

During the next 6 months, he gradually developed shortness of breath and stridor, and eventually presented to the emergency department in respiratory distress requiring intubation for 2 days

Shortly after extubation, laryngeal examination showed fullness of the posterior pharyngeal wall and severely limited vocal fold abduction bilaterally (Figure 1A

A modified barium swallow study showed intermittent laryngeal penetration without aspiration (Video 2)

Although the patient was unable to tolerate magnetic resonance imaging, a soft tissue computed tomography scan of the neck with contrast revealed a well-positioned plate with no evidence of fluid collection in the setting of a normal leukocyte count

One month later, a neuromuscular specialist investigated potential underlying neurological causes

however, results of nerve conduction, antiacetylcholine receptor, antimuscle-specific kinase, methylmalonic acid, HIV, rapid plasma reagin, vitamin B-12, thyroid stimulating hormone, thyroxine, copper, creatine kinase, and immunofixation studies were all unremarkable

Maximum abduction of vocal folds on laryngoscopic examination 11 months after placement of anterior cervical spinal plate (A) and 2 months after removal (B)


Blastic plasmacytoid dendritic cell neoplasm

Cutaneous angioimmunoblastic T-cell lymphoma

A 61-year-old man presented to our clinic with a 3-month history of rapidly growing and itchy cutaneous nodules all over the body

He denied systemic complaints such as weakness, fever, night sweats, or weight loss

Oral glucocorticoids did not show any therapeutic effect

His medical history was remarkable for mild anemia because of untreated hemorrhoids

Physical examination revealed numerous symmetrically distributed, well-defined, erythematous skin nodules on his back, buttocks, and limb extensors (Figure, A)

Prominent lymphadenopathies on bilateral cervical, axillary, and inguinal lymph nodes were noted, with the largest lymph node measuring approximately 5 × 2 cm

Laboratory tests were significant for atypical lymphocytes in the peripheral blood (count up to 6%), an elevated CD4+/CD8+ lymphocyte ratio (9.21

normal range, 0.7-3.1), moderate anemia (hemoglobin level, 8.7 g/dL

normal range, 11.5-15.0 g/dL [to convert to g/L, multiply by 10.0]), a high erythrocyte sedimentation rate (35 mm/h

normal range, 0-25 mm/h), and cryoglobulins composed of mixed polyclonal immunoglobulins

normal range, 125-350 × 103/μL [to convert to ×109/L, multiply by 1.0]) and lactate dehydrogenase (217 U/L

normal range, 100-240 U/L [to convert to μkat/L, multiply by 0.0167]) were normal

Quantification of Epstein-Barr virus (EBV) DNA showed high loads of virus in the peripheral blood (846 copies/mL in serum, 27 700 copies/mL in lymphocytes

A biopsy specimen from a skin nodule was sent for histopathologic examination (Figure, B)

A, Physical examination showed widespread, symmetrically distributed, well-defined nodules on the trunk and extremities

B, Skin biopsy sample showed infiltration of atypical lymphocytes and histiocytes with proliferation of high endothelial venules (hematoxylin-eosin)

C, In situ hybridization was positive for Epstein-Barr virus in the skin


A man in his 80s with a medical history of gastric adenocarcinoma treated with total gastrectomy presented with asymptomatic lesions on his tongue that gradually developed during the previous 3 months

Intraoral examination revealed multiple yellowish to erythematous papules and verrucous cobblestone-like plaques on the mucosal surface on both sides of his tongue (Figure, A and B)

The skin of the face, neck, arms, and other sun-exposed areas did not reveal any associated lesions

He reported occasional alcohol intake, and there was no history of tobacco-related habits or oral trauma

The results of laboratory investigations, including a complete blood cell count, serum chemistry panel, liver and kidney function test, C-reactive protein levels, lipid profile, and serum and urine electrophoresis disclosed no abnormalities

With the clinical suspicion of squamous cell carcinoma, an incisional biopsy was performed (Figure, C and D)

Yellowish plaques in a cobblestone pattern on the right (A) and left (B) side of the tongue

C and D, Circumscribed eosinophilic, amorphous fissured masses in the subepithelial connective tissue (hematoxylin-eosin)


A man in his 70s presented with a 6-month history of a rash on the left forearm, which gradually increased in severity

The patient developed a rash on the dorsal aspect of his hand half a year ago without any pain or itching discomfort and denied any history of injury or exposure to suspected pathogens prior to onset

After self-medication with topical herbal medicine, the lesions gradually spread to the forearm and fused into plaques, and ulcers often recurred on the surface

He denied any systemic symptoms and concerns

Physical examination revealed large, firm, mildly swollen erythematous plaques on the back of the left forearm with purulent discharge and crusting (Figure, A)

Results of routine laboratory investigations, including complete blood cell count, biochemical profile, and chest radiography, were normal

A biopsy specimen was taken for pathological examination (Figure, B-D)

A, A large, firm, mildly swollen erythematous plaque with purulent discharge and crusting on the back of the left forearm

B, Hyperkeratosis, pseudoepitheliomatous hyperplasia, and lymphohistiocytic infiltrate with neutrophils, plasma cells, and a small amount of multinucleated giant cells in the dermis (hematoxylin-eosin)


A 66-year-old woman with chronic hepatitis B infection and hepatocellular carcinoma (HCC) presented with subacute epigastric pain

Three years prior, she was diagnosed with a solitary HCC and underwent surgical resection with curative intent

Pathology revealed poorly differentiated HCC, 16 cm in largest dimension, with large vein involvement (T3bNXM0, R0 resection)

The patient underwent expectant observation

One and a half years prior to the current presentation, laboratory results revealed an elevated α-fetoprotein (AFP), and cross-sectional imaging revealed multiple lung nodules consistent with metastases

She was treated with nivolumab every other week and attained a biochemical (baseline, 212.2 ng/dL

best response, 3.3 ng/dL) and radiographic complete response

In the current presentation, the patient reported nonradiating epigastric pain of moderate intensity for 1 month

Associated symptoms included nausea, anorexia, and water brash without emesis

Review of systems was positive for unchanged chronic abdominal bloating and negative for other constitutional or gastrointestinal symptoms

Laboratory results, including liver function tests, amylase, lipase, and AFP, were within normal limits, and hepatitis B virus polymerase chain reaction was undetectable

Computed tomography with multiphasic liver protocol showed new enlarged mesenteric nodes and diffuse thickening of the gastric body (Figure, A)

Esophagogastroduodenoscopy (EGD) identified diffuse inflammation and ulcerations in the gastric body and antrum (Figure, B)

Biopsy specimens showed chronic and active mixed inflammatory infiltrate, including intraepithelial lymphocytosis (Figure, C)

No overt viral cytopathic effects were observed, and immunostaining results for Helicobacter pylori, cytomegalovirus, and adenovirus were negative

A, Coronal view of restaging computed tomography of chest, abdomen, and pelvis with contrast

The yellow circle notes borderline enlarged mesenteric nodes inferior to the stomach, and the yellow arrowheads show thickened gastric body wall

B, Gross appearance of the gastric mucosa visualized via upper endoscopy, which showed diffuse inflammation and ulceration in the gastric body and antrum

C, Biopsy of the gastric mucosa showed marked chronic and active intraepithelial infiltration of lymphocytes (blue arrowheads) and neutrophils (yellow arrowhead) and associated glandular epithelial injury with withered glands and gland dropout (red circle) (original magnification ×100)


A 65-year-old patient was referred to the ear, nose, and throat department with left-sided hearing loss during the past 6 months without evident cause

He had a medical history of myocardial infarction, sleep apnea, Ménière disease, and congenital cataract

Furthermore, he did not report otalgia, otorrhea, a recent episode of Ménière disease, or other ear, nose, and throat or systemic symptoms

The patient used atorvastatin and acetylsalicylic acid, did not smoke, and reported drinking 2 glasses of wine daily

Otoscopy of the left ear revealed a yellow-whitish calcareous lobular mass located in the mesotympanum anterior to the malleus with an intact tympanic membrane

The lesion was hard and painful during palpation (Figure 1A)

Otoscopy results for the right side were normal

Conductive hearing loss of the left ear because of a middle ear mass, seen on otoscopy (A) and on computed tomography (CT) (B)

Pure-tone audiometry results showed a left-sided mixed hearing loss with a pure tone average of 110 dB HL and bone conduction levels at approximately 60 dB HL

The tympanogram showed a normal A-type curve with a normal to low middle ear compliance of 0.5 mm on the left side

Computed tomography (CT) imaging of the middle ear showed a heterogeneous hyperdense round mass of 5.5 mm in the left middle ear with a close relation to the malleus, eardrum, and tegmen (Figure 1B)

There were no other abnormalities in the mastoid, ossicular chain, facial nerve canal, or surrounding blood vessels


A 6-month-old girl presented to the emergency department for evaluation of fever and was noted to have mild inspiratory stridor

Her parents stated that her stridor began acutely at 4 months of age without any inciting illness or event

The stridor was worse with agitation but was without positional exacerbation

The family reported that the stridor had remained stable since onset

Her parents noted mild dysphagia but denied shortness of breath, cyanosis, or apnea

Laboratory test results revealed acute COVID-19 infection

Physical examination was unremarkable other than mild inspiratory stridor

Findings from bedside flexible laryngoscopy revealed intact vocal fold motion bilaterally with glottic competence

No supraglottic or glottic masses were identified

Anterior-posterior neck and chest radiography demonstrated narrowing and tortuosity of the trachea with displacement to the right proximal to the thoracic inlet and diffuse patchy heterogenous airspace opacities (Figure, A)

Subsequent neck ultrasonography and computed tomography scan demonstrated a 3.2 × 2.0-cm low-density lesion abutting the trachea and hypopharynx at the level of thoracic inlet, with rightward displacement of the trachea and larynx (Figure, B-D)

Operative laryngoscopy, bronchoscopy, and esophagoscopy revealed extrinsic compression of the trachea, right mainstem bronchus, and esophagus with rightward deviation of the trachea and esophagus

A, Narrowing and tortuosity of the trachea with rightward deviation on anterior-posterior neck radiograph (arrowhead delineates tracheal deviation)

B, Echogenic cystic lesion on ultrasonogram

C and D, Low-density lesion abutting the trachea and pharynx with rightward deviation of the trachea and larynx on coronal and axial neck computed tomography (black arrowheads delineate tracheal deviation


A 15-year-old girl presented to the otorhinolaryngologic department for a right-side postauricular mass

The mass had been present since birth but had slowly grown larger in the previous 2 years

On physical examination, a 2.5 × 1.5-cm, nontender, blue, soft, cystic mass was observed in the right postauricular area

There were no fistular openings around the auricle or in the external auditory canal (EAC), and there were no additional cysts, sinuses, or fistulas at the right neck from the hyoid bone and chin to the EAC

On temporal bone computed tomography, a 2.5 × 1.5-cm, hypodense, oval-shaped cystic mass was revealed at the subcutaneous fat layer superoposterior to the right auricle

On temporal magnetic resonance imaging (MRI), an oval cystic mass was revealed at the same location, with high signal intensity on both T1-weighted and T2-weighted images and low signal intensity on enhanced T1-weighted fat suppression and T2-weighted short T1 inversion recovery images (Figure, A-D)

In surgery, the well-demarcated cystic mass was completely excised

Yellowish fluid leaked out of the cyst during excision

On histopathologic examination, the cyst was lined by keratinizing stratified squamous epithelium with hair follicles and sebaceous glands

The cyst contained keratinous debris (Figure, E)

There was no recurrence 6 months after surgery

A-D, Magnetic resonance imaging showed a 2.5 × 1.5-cm, oval cystic mass at the subcutaneous fat layer superoposterior to the right auricle with high signal intensity on both T1-weighted and T2-weighted images and low signal intensity on enhanced T1-weighted fat suppression and T2-weighted short T1 inversion recovery images (in order from the left)

E, Hematoxylin-eosin staining showed a cyst composed of keratinizing stratified squamous epithelium, hair follicles, and sebaceous glands


A 28-year-old White woman with no remarkable medical history presented with difficulty phonating and singing for the past 15 months

She also reported odynophonia, globus sensation, and mild dysphagia to solids

The patient had been treated previously with steroids and amoxicillin for the presumptive diagnosis of chronic laryngitis but reported no improvement in symptoms

Laboratory workup was positive for elevated antinuclear antibodies and erythrocyte sedimentation rate and was consequently referred to our institution

On presentation, the patient had only minimal dysphonia

She underwent a transnasal flexible laryngoscopy that demonstrated a severely edematous epiglottis, posterior pharyngeal wall, aryepiglottic folds, and arytenoids (Figure, A)

The vocal folds were difficult to visualize but seemed normal in appearance and mobility

Stroboscopy could not be performed because of obstruction from the prolapsing arytenoids

Interestingly, the patient denied any shortness of breath

Results of laboratory testing revealed normal levels of all components of a comprehensive metabolic panel, thyroid-stimulating hormone, and angiotensin-converting enzyme

The patient’s erythrocyte sedimentation rate was elevated to 75 mm/h (reference range, 0-15 mm/h)

Findings of chest radiography were normal

An esophagram showed no evidence of reflux esophagitis and a computed tomography of the neck showed edema of the supraglottis with some edema in the posterior hypopharyngeal wall

A, Awake flexible laryngoscopy image of the edematous epiglottis, arytenoids, and posterior pharyngeal wall

B, High-magnification of hematoxylin-eosin staining reveals well-formed noncaseating granulomas (arrowheads) with lymphoid reaction visible around the granulomas

Subsequently, the patient underwent a direct laryngoscopy with laryngeal biopsy

Pathology results demonstrated hyperplastic squamous mucosa with submucosal chronic inflammation and fibrosis and were negative for high-grade dysplasia and carcinoma

scattered nonnecrotizing granulomas in the subepithelium were also observed (Figure, B)


An otherwise healthy man in his 60s presented to our department with an 8-month history of widespread pruritic papulonodular skin lesions on his extremities and trunk

The patient had been initially diagnosed with nodular prurigo at a local hospital and treated with various topical steroids and oral antihistamines for 2 months, without significant improvement

The skin lesions continued to progress and worsened after scratching

Physical examinations revealed disseminated erythematous papules, hypertrophic plaques approximately 1 to 2 cm in diameter, and dome-shaped nodules with excoriated whitish or pink centers on the scalp, chest, abdomen, back, waist, and extremities (Figure, A and B)

Subsequent examinations of the mucosal membranes appeared normal

Other physical examinations as well as his medical and family histories were unremarkable

Peripheral eosinophils, serum total immunoglobulin E level, enzyme-linked immunosorbent assay (ELISA) test for HIV, and results of other routine laboratory tests were within normal ranges or negative

A biopsy specimen was taken from one of the skin lesions on his right forearm for histopathological examination and direct immunofluorescence (DIF) testing (Figure, C and D)

A and B, Clinical images showed disseminated erythematous papules, hypertrophic plaques approximately 1 to 2 cm in diameter, and dome-shaped nodules with excoriated whitish or pink centers on the back, waist, and extremities

C, A biopsy specimen taken from the right forearm showed marked hyperkeratosis, acanthosis with irregular elongation of the rete ridges, fibrotic dermis, and perivascular lymphocytic infiltrate (hematoxylin-eosin)

D, Direct immunofluorescence revealed linear deposition of immunoglobulin G along the basement membrane zone


Multifocal lymphangioendotheliomatosis with thrombocytopenia

Congenital disseminated pyogenic granuloma

A 3-month-old boy was referred for evaluation of multiple cutaneous red papules

The guardian reported first noticing the papules at birth

During the following months, the papules grew in number and size, involving the head and neck, trunk, extremities, right palm, mons pubis, and buttocks

At the height of severity, the patient had 39 lesions, some of which were associated with ulceration and bleeding

The patient was born at full term via spontaneous vaginal delivery

the birth mother had not received prenatal care

Neither the birth mother nor the infant had any known history of environmental or occupational exposure to hazardous substances

The paternal family medical history was unremarkable

maternal family medical history was unknown

Physical examination revealed numerous bright red macules, papules, and plaques ranging in size from 1.0 × 1.0 mm to 2.2 × 2.3 cm, many of which had hemorrhagic crust and dark scale (Figure, A and B)

Additional examination findings included retrognathia, thickened maxillary frenulum, and anterior ankyloglossia

A, Irregularly shaped red vascular plaque (2.2 × 2.3 mm) with overlying scale, dark crusting, and a dark blue component with surrounding (1.0-3.0 mm) red papules

B, Numerous bright-red macules and papules (1 × 1 mm-3 × 3 mm) and 1 vascular red nodule (1.6 × 1.1 cm) on the left lateral neck with dark overlying crusting

C, Low-power view of histopathology findings demonstrating epidermal hyperplasia and an upper dermal vascular proliferation (hematoxylin-eosin section)


Posterior communicating artery aneurysm that is not picked up on CT angiography

Neurovascular conflict of the oculomotor nerve

A 37-year-old Asian nonsmoking man with no known medical or drug history presented with an insidious onset of left-eye ptosis (only brought to attention by his friends on review of recent photographs) and subsequent painless vertical diplopia for 1 year

He did not complain of headaches, facial numbness, dysarthria, facial droop, dysphagia, or limb weakness or numbness

There was no history of joint pain or constitutional symptoms

Neurological examination revealed a nonfatigable partial left-eyelid ptosis, left-eye hypotropia at primary position, and impaired left eye supraduction (Figure 1) with a nonreactive left pupil on direct and consensual light reflexes

There were no other cranial nerve deficits

Limb reflexes, motor testing, and sensory testing results were normal, and the plantar responses were flexor

Top left (looking right and up), middle left (looking right), bottom left (looking right and down), center (primary position), top right (looking left and up), middle right (looking left), and bottom right (looking left and down)

The images demonstrate left-eye hypotropia and left-eyelid ptosis at the primary position and impaired left-eye supraduction worse at lateral gaze on extraocular movements

Laboratory evaluation showed a normal fasting serum glucose level, thyroid function test result, and erythrocyte sedimentation rate

Anti–double-stranded DNA, antinuclear antibodies, and HIV test results were negative

Repetitive nerve stimulation did not show any decremental response and anti-acetylcholine receptor antibodies were negative

A computed tomography (CT) angiography of the circle of Willis did not show any aneurysms and a routine magnetic resonance imaging (MRI) of the brain did not show any infarcts or masses

Lumbar puncture was acellular with normal glucose and protein levels, and multiple microbiological test results were negative


An 81-year-old woman presented with a 3-month history of a slow-growing mass in her left cheek

She denied having any symptoms, tenderness, numbness, or facial nerve weakness

Physical examination revealed a mass in the buccinator space that was smooth, was nontender to palpation, and had no overlying cutaneous changes

A contrast-enhanced maxillofacial computed tomography scan demonstrated a 1.8 × 1.8 × 1.9-cm rounded, well-circumscribed lesion at the anterior aspect of the left masticator space (Figure 1)

The mass was abutting the parotid duct, which did not appear dilated, and appeared to have a peripheral rim of hyperenhancing soft tissue with a central cystic/necrotic component

Results of ultrasound-guided fine-needle aspiration revealed rare epithelial cells with degenerative changes and inflammatory cells present

Findings from a core biopsy demonstrated a spindle cell neoplasm of uncertain origin

On immunostaining, spindle cells were CK8/18, S100, and SOX10 negative

myosin and smooth muscle actin (SMA) negative

However, STAT6 staining was diffusely positive

Axial view of the contrast-enhanced maxillofacial computed tomography scan demonstrating the left cheek mass (arrowhead)


A 21-year-old man was referred to our tertiary care hospital with a congenital left hemifacial weakness and a chronic unilateral left-sided hearing loss that evolved since birth

On audiometry, normal hearing sensitivity in the right ear and a severe to profound sensorineural hearing loss with poor word recognition score in the left ear were noted

On physical examination, the patient presented a grade VI peripheral left facial paralysis

Findings from the rest of the clinical examination were within normal limits

Results of cerebral magnetic resonance imaging (MRI) and temporal bone computed tomography (CT) revealed a left internal auditory canal mass, measuring 13×12 mm, developed at the expense of nerve VII, with a dilation of the various portions of the facial nerve with extension to its distal branches

It presented a hypointensity on T2, isointensity on T1, and was strongly enhanced after the injection of gadolinium

This mass was responsible for the enlargement of the internal auditory canal and was in close contact with the left middle cerebral fossa, without intraparenchymal extension (Figure 1A and B)

A, Cerebral MRI T1-weighted contrast-enhanced image showing the mass

B, Temporal bone CT scan showing the mass

Arrowheads indicate the abnormal facial nerve

CT denotes computed tomography, and MRI, magnetic resonance imaging


A woman in her late 70s with a history of immunoglobulin A monoclonal gammopathy of unknown significance presented with a tender, draining lesion of the central face despite several courses of treatment with antibiotics (Figure, A)

The patient lived in Southern California but had visited Kenya for an extended trip a few years prior

Review of systems was negative for fevers, night sweats, weight loss, headache, vision changes, respiratory distress, or arthralgias

On the left nasal dorsum and medial cheek, there was a 3 × 4 cm violaceous plaque with focal purulent drainage

Within several weeks, the lesion rapidly expanded into a vegetative, freely draining plaque covering the cheeks and glabella and approaching the medial canthi (Figure, B)

Computed tomography revealed findings consistent with abscess that did not involve the orbits, sinuses, or bone

Chest radiography results were unremarkable

Punch biopsy specimens were obtained for histopathological analysis and tissue cultures (Figure, C)

Tissue culture results at 2 weeks remained negative, and a QuantiFERON-TB Gold test result was negative

A, Violaceous plaque on the nose and left cheek

B, Rapid progression of the lesion involved the cheeks and glabella, with substantially worsening drainage at 6-week follow-up

C, Punch biopsy of lesion showing suppurative and granulomatous dermatitis (hematoxylin-eosin)

D, Posttreatment, there was improvement of the lesion from initial presentation, but substantial residual facial scarring remained


A 31-year-old man with insidious onset of limited mobility of joints visited our clinic for evaluation

Six months prior, he found his palms could not make full contact with each other (the “prayer sign”)

He had not been able to fully clench his fists for 2 months or squat for the past 10 days

No erythema, edema, or sclerotic skin plaque had been found on his limbs or trunk

His medical and family histories were unremarkable

On examination, he showed bilateral limited mobility of interphalangeal (hands), metacarpophalangeal, wrist, interphalangeal (feet), metatarsophalangeal, and ankle joints (Video)

No muscular atrophy or sclerodactyly was found

The prayer sign (Figure 1A) and the “groove sign” (Figure 1B) were observed

Blood test results indicated eosinophilia (absolute eosinophil count, 1300 μL

reference, <500μL [to convert to ×109/L, multiply by 0.001]), elevated erythrocyte sedimentation rate (ESR, 31 mm/h

reference, <15 mm/h) and C-reactive protein (CRP, 3.01 mg/dL

reference, <0.8 mg/dL [to convert to mg/L, multiply by 10])

Liver and kidney function tests, creatine kinase level, electrolyte level, thyroid function, globulin concentration, and antinuclear antibody (ANA) level were unremarkable

Electrocardiogram, cervical spine magnetic resonance imaging, and plain radiographs of hands, wrists, feet, and ankles were normal

Nerve conduction studies performed in all 4 limbs were normal

Electromyography (EMG) showed spontaneous activities in muscles of limbs, including fibrillation potentials and positive sharp waves (1+ or 2+)

Myotonic discharges were seen in bilateral extensor digitorum

A, The patient’s bilateral palms and fingers could not make full contact due to the limited mobility of interphalangeal and metacarpophalangeal joints

B, Linear depression (arrowheads) was seen along the course of superficial vein when limb was elevated


Intravascular anaplastic large cell lymphoma

A 79-year-old female patient presented with a 2-year history of asymptomatic erythematous-infiltrated patches and plaques on the right side of her scalp and face

The lesions gradually enlarged and coalesced into large plaques and nodules

Mild erosions occurred occasionally on the surface of the lesions

She denied having fever, fatigue, chills, night sweats, or weight loss

Physical examination demonstrated ill-defined, indurated erythematous-violaceous patches, nodules, and plaques involving the surface of her head and face with sporadic erosion and brownish crusts (Figure, A)

The findings of systemic reviews were unremarkable, and there was no lymphadenopathy

The complete blood cell count revealed normal white blood cell count

The biopsy from an erythematous nodule displayed sheets of medium to large mononuclear cells infiltrated in the dermis, some of which filled and expanded apparent dermal blood vessels and had large, irregular, pleomorphic nuclei (Figure, B)

In addition, some neoplastic cells formed a balloon appearance

The initial immunohistochemical stain revealed the tumor cells were positive for CD3 and negative for CD20, AE1/AE3, CK20, chromogranin A, and melan-A

A, Ill-defined, indurated erythematous-violaceous patches, nodules, and plaques on the head and face with sporadic erosion and brownish crusts

B, The biopsy showed dilated vessels expanded by cohesive clusters of large atypical lymphoid cells (hematoxylin-eosin staining)

C, Immunohistochemical staining of D2-40 highlighted the exclusively lymphovascular location of the lymphoma


A 66-year-old African American man presented to the otolaryngology clinic with a several-month history of severe nasal congestion

Five years before this otolaryngology evaluation, the patient developed diffuse skin plaques with erythema and was subsequently diagnosed with hypopigmented cutaneous T-cell lymphoma (CTCL, mycosis fungoides type)

The patient was treated with a regimen of narrowband UV-B phototherapy and triamcinolone cream, which he has continued for 4 years

Owing to significant worsening of his symptoms 4 months before his evaluation, a biopsy of his cutaneous lesions was performed to rule out progression of CTCL (Figure 1)

Physical examination demonstrated large annular plaques with peripheral hyperpigmentation of the body

There were also confluent, indurated pink plaques with slightly raised edges along the forehead, malar eminence, and scalp

The nasal septum demonstrated an inverted V deformity with compromise of the keystone junction

Nasal passages were obstructed with thick mucus and synechiae formation, nearly obstructing the entire nasal passage bilaterally

A, Fite acid-fast stain of cutaneous lesion identifying pink bacilli (original magnification ×100)

B, Hematoxylin-eosin stain of the same cutaneous lesion showing poorly formed granulomas (original magnification ×40)


A 29-year-old Pakistani woman was referred to the otolaryngology clinic for increased postnasal drip, pharyngitis, odynophagia, cervicalgia, cervical lymphadenopathy, and right-sided otalgia that had persisted for more than a week

She was breastfeeding her 10-month-old, had no history of alcohol use, and had a remote history of hookah use

She was a Southern California native with travel history to Pakistan, Turkey, Jordan, Israel, Qatar, and Dubai 5 years prior to symptom onset

One month prior to onset, she drove from Los Angeles, California, to Houston, Texas

She denied recent unintentional weight loss, cough, fever, night sweats, chills, stridor, dyspnea, dysphonia, or rash

On examination, her vital signs were within normal limits, and physical examination revealed a small right tonsillolith, mild tonsillar erythema that was worse on the right side, and a mobile 2-cm, right level 2 lymph node that was slightly tender to palpation

Laboratory evaluation was notable for an elevated eosinophil count and positive Epstein-Barr immunoglobulin G

Her white blood cell count and mononucleosis spot test result were within normal parameters

Her symptoms persisted despite treatment with penicillin, azithromycin, and steroids from her primary physician and amoxicillin-clavulanate, sinus rinses, fluticasone propionate, and azelastine spray from an otolaryngologist to treat any contributing postnasal drainage and tonsillitis

Flexible transnasal videolaryngoscopy revealed a lesion on the laryngeal surface of the right epiglottis passing midline extending to the right aryepiglottic fold, but not involving the true vocal cords (Figure 1A)

Given inconclusive office biopsy results, direct laryngoscopy with biopsy was performed, showing extensively inflamed squamous mucosa with ulceration, granulation tissue, pseudoepitheliomatous hyperplasia, and atypia (Figure 1B)

A, In-office laryngoscopy showing an ulcerative, papillomatous lesion on the laryngeal surface of the right epiglottis that passed midline and extended to the right aryepiglottic fold but did not involve the true vocal cords

B, Laryngeal biopsy specimen demonstrating extensively inflamed squamous mucosa with ulceration, granulation tissue, pseudoepitheliomatous hyperplasia, and atypia, with background chronic granulomatous inflammation


An otherwise healthy man in his 60s presented to a tertiary otolaryngology clinic reporting a 1-year medical history of progressively worsening hoarseness and dyspnea

He denied any history of dysphagia, odynophagia, fevers, unintentional weight loss, cough, or reflux symptoms

There was no prior history of smoking, head and neck surgeries, or radiation exposure

He was treated for asthma with inhaled steroids by his primary care physician without improvement in symptoms

On physical examination, the patient was stridulous and considerably hoarse

Subsequent videostroboscopy revealed severe laryngeal mucosal edema, most significant in the false vocal folds, interarytenoid, and postcricoid regions (Figure 1A)

There was right true vocal fold immobility with compensatory squeeze of the false vocal folds and an absent mucosal wave

Computed tomographic (CT) scan of the neck with contrast showed irregularity along the free edges of the true vocal folds but no evidence of a neoplasm or cervical lymphadenopathy

The patient underwent urgent direct laryngoscopy with biopsy, which showed edematous, irregular, friable laryngeal mucosa with significant airway narrowing

Laryngeal biopsy findings are shown in Figure 1, B and C


Farber disease (disseminated lipogranuloma)

A 2-year-old female toddler born to nonconsanguineous parents presented with multiple skin-colored subcutaneous nodules that developed 40 days after birth

The lesions started on the left foot and subsequently progressed to involve the face, trunk, and extremities, with distal interphalangeal joint deformity of both hands (Figure, A)

The child had an older sibling with no skin ailments, and no other family members were affected

Parents reported decreased oral intake, decreased activity, and delayed motor development

A, Nodules on the right hand and joint deformity

B and C, Histopathological images show a granulomatous pattern, numerous foamy cells, marked collagen degeneration, and mucin deposition (hematoxylin-eosin stain)

D, Comma-shaped curvilinear tubular structures (yellow arrowhead)

On examination, dozens of tough to hard subcutaneous skin-colored nodules ranging from 0.5 to 5 cm in diameter were observed on both sides of the nose and parts of both wrists, the knees, elbows, ankles and hips, and the trunk

Symmetrical beaded subcutaneous nodules were observed in the bilateral auricles, while the mucous membrane was not involved

The child weighed 8 kg (normal, 13-15 kg) and was 87 cm (normal, 91-95 cm) in length

In addition, a physical examination revealed horizontal nystagmus and voice hoarseness

The distal interphalangeal joints of both hands were stiff with limited extension

The interphalangeal joints of the third through fifth distal segments of both feet were slightly bent

A complete hematological and biochemical survey, including liver and renal function, C-reactive protein levels, thyroid function, rheumatoid factor, and antinuclear antibodies, identified no abnormalities

A magnetic resonance imaging study of the brain showed volume loss in the parietooccipital region

Ultrasonographic evaluation of metacarpophalangeal joints showed multiple hypoechoic nodules on the skin of the hands

Radiography showed deformities of the interphalangeal joints with soft tissue swelling

A punch biopsy was obtained from 1 of the nodules (Figure, B-D)


A man in his 60s with anti-RNA polymerase III antibody–positive systemic sclerosis complicated by scleroderma kidney crisis, heart failure, interstitial lung disease, sclerodactyly, and Raynaud phenomenon while receiving mycophenolate mofetil, 1 g twice daily, presented with a pruritic rash that had been present for 3 months

The patient denied any known preceding triggers or trauma to the area of the rash

He had tried treating with topical aloe, without improvement

Review of systems was negative for any new or worsening constitutional, respiratory, or gastrointestinal symptoms

The patient’s medical history was otherwise unremarkable

Examination findings were notable for firm, red-brown plaques without overlying scale on the proximal upper extremities and anterior trunk (Figure, A and B)

There was no appreciable skin tightening in the affected areas

Complete blood cell count with differential was unremarkable

Lesional punch biopsy specimens were obtained (Figure, C and D)

A, Clinical image of firm, red-brown plaques without overlying scale on the proximal upper extremities and anterior trunk

B, Clinical image of nodular plaques on the right shoulder and chest

C and D, Lesional punch biopsy specimens (hematoxylin-eosin)


A 75-year-old woman presented with horizontal binocular diplopia, right-sided ptosis, and a new headache that was progressive over 3 days

She reported difficulty opening her jaw, pain when chewing, and a 2.3-kg weight loss

The week prior, she experienced left-sided ptosis that persisted for 2 days and subsequently resolved

She denied vision changes, eye pain, scalp tenderness, and myalgias

Past medical history included schizophrenia, hypothyroidism, and supraventricular tachycardia

Her examination demonstrated normal visual acuity in both eyes

Extraocular motility demonstrated limitation of elevation and abduction bilaterally (Figure 1)

Results of dilated fundus examination were normal, and the remainder of her neurologic examination results were normal

Her eye movements and ptosis did not change with fatiguability, rest, or an ice-pack test

Investigations revealed a C-reactive protein level of 292 mg/L (normal, <10 mg/L

to convert to milligrams per deciliter, divide by 10) and erythrocyte sedimentation rate of 93 mm/h (normal, <30 mm/h)

A lumbar puncture demonstrated normal opening pressure, cell count, and glucose and protein levels

Magnetic resonance imaging with contrast of the brain demonstrated diffuse edema and enhancement of the scalp, skull base, neck, and paraspinal soft tissues, as well as T2 hyperintensity and enhancement of the extraocular muscles bilaterally

An additional test was obtained, and a diagnosis was made

Examination reveals deficits in elevation and abduction of both eyes not conforming to a cranial nerve distribution


A 32-year-old Black woman presented with a 1-year history of facial swelling, predominantly in the right preauricular and left submandibular regions

Her symptoms began after relocation to California and occurred more frequently at her home

However, she could not discern any environmental or dietary triggers

She reported pruritus and swelling, which were usually moderate but occasionally severe, prompting several emergency department visits

During an emergency department visit, she was diagnosed with acute parotitis based on clinical and radiographic findings of diffuse gland edema, without salivary stones or masses

Her symptoms responded to oral prednisone bursts and diphenhydramine, but not to second-generation antihistamines or montelukast

Her workup included immune serology and total immunoglobulin (Ig) E measurements, all of which were within normal limits

A differential blood cell count revealed 10% eosinophils (normal, <6%) and an absolute eosinophil count 500/µL (to convert to ×109/L, multiply by 0.001)

Titers for antinuclear antibodies, rheumatoid factor, anti-Ro (SS-A), and anti-La (SS-B) were negative

Sialendoscopy of her right parotid and left submandibular glands found no obstructing stones or strictures

Concurrent steroid irrigation did not provide any substantial benefit

An open biopsy of the right parotid tail revealed nonspecific inflammatory changes and otherwise normal parenchyma

Given the frequency and severity of her symptoms along with multiple treatment failures, she opted to undergo a right superficial parotidectomy, which proceeded without complications

Further histologic examination of the surgical specimen revealed substantial periductal inflammatory infiltrate with eosinophils, as seen in the Figure

Medium-powered (A) and high-powered (B) field from the superficial parotid gland (hematoxylin-eosin)


A 2-month-old, full-term female presented with a left upper neck mass that was first noted at birth

Her parents reported no fevers, constitutional symptoms, or enlargement of the mass

On physical examination, there was a nontender 4-cm mass in the left submandibular region with no overlying skin changes

Ultrasonography results showed an avascular cystic structure with internal debris and a single thin septation

Contrast-enhanced computed tomography (CT) of the neck soft tissue revealed a 3-cm low attenuation, nonenhancing, cystic-appearing mass that elevated the submandibular gland to the level of the parapharyngeal fat space with medial extension of the lesion into the tongue musculature

The walls were thin except for mildly thickened capsule along the posterior aspect of the lesion (Figure, A and B)

Fluid that aspirated from the cyst was serous, and cytology showed mature and anucleate squamous cells with acute inflammation

Further analysis 8 months after the initial CT via contrast-enhanced magnetic resonance imaging (MRI) showed a large, multiseptated cystic structure (Figure, C)

the capsule of the wall was mildly thickened and was contrast enhancing

At age 12 months, the mass was excised via neck dissection

A lateral component at the level of the sternocleidomastoid muscle was completely excised

An intraoperative rupture of the thin capsule caused some spillage of cystic contents that were milky white in some areas and mucinous in others

Contrast-enhanced computed tomography images

A, Axial: the blue arrowhead indicates extension into the tongue musculature, the yellow arrowhead indicates focally thickened capsule posteriorly, and the white arrowhead indicates a displaced sternocleidomastoid muscle

B, Coronal: the black arrowhead indicates an elevated left submandibular gland, and the white arrowhead indicates a normal right submandibular gland

C, Axial T2-weighted magnetic resonance image showing marked interval enlargement of the multiseptated cystic mass

Arrowheads indicate involvement of the tongue musculature


An otherwise healthy man in his early 70s presented to the Department of Dermatology with a 2-year history of a mildly pruritic, erythematous, and gradually enlarging lesion on the proximal portion of his left calf

The lesion had appeared without an identifiable cause, and for the previous 2 months, the patient had applied topical corticosteroids and antifungals with no improvement

Physical examination revealed an ill-defined, nonindurated, erythematous-violaceous plaque that was slightly raised but with an atrophic appearance (Figure, A)

A skin biopsy was obtained for histopathology examination (Figure, B-D)

A, Clinical image of the left calf showing an ill-defined, erythematous-violaceous plaque with an atrophic appearance

B, Band-like vascular proliferation in the upper dermis with no grenz zone (hematoxylin-eosin)

C, Round vascular channels lined by a single layer of endothelial cells without atypia (hematoxylin-eosin)

D, CD34 immunostaining labels endothelial cells


A White man in his 70s with a medical history notable only for mitral valve repair was incidentally found to have cervical lymphadenopathy on magnetic resonance imaging while undergoing workup for shoulder pain

A fine-needle biopsy of an enlarged left-sided cervical lymph node yielded benign results

One month later, he presented to a local emergency department with diffuse abdominal pain

Symptoms began 6 weeks earlier and had been intermittent in nature

He had also experienced fatigue, night sweats, decreased appetite, and weight loss for 6 weeks

He otherwise denied having fevers, myalgias, nausea, vomiting, diarrhea, or easy bruising

Physical examination revealed bilateral cervical, axillary, and inguinal lymphadenopathy in addition to mild diffuse abdominal pain

Laboratory results revealed a hemoglobin level of 13.1 g/dL (to convert to g/L, multiply by 10), a white blood cell count of 6.4 × 103/μL, and a platelet count of 404 × 103/μL  (to convert to × 109/L, multiply by 1) in addition to negative results for HIV, hepatitis B virus, and hepatitis C assays

Additional workup results were notable for elevated levels of interleukin (IL) 10 (1091 pg/mL), IL-6 (3.1 pg/mL), C-reactive protein (4.9 mg/dL [to convert to mg/L, multiply by 10]), and erythrocyte sedimentation rate (79 mm/h)

A computed tomography scan of the neck, chest, abdomen, and pelvis further revealed extensive cervical, supraclavicular, retroperitoneal, pelvic, and inguinal lymphadenopathy, with extensive inflammation and fat stranding adjacent to these enlarged nodes

Positron emission tomography–computed tomography further demonstrated fluorodeoxyglucose-avid lymph nodes above and below the diaphragm

The patient then underwent excisional biopsy of an enlarged right cervical lymph node (Figure)

A, Proliferation of slitlike blood vessels (hematoxylin-eosin)

B, The lymphoid follicle shows regression, with onion-skinning of mantle zone lymphocytes and hypervascularity (hematoxylin-eosin)

Scattered atypical plasma cells are present


Primary cutaneous embryonal rhabdomyosarcoma

An adolescent girl presented with an asymptomatic slightly reddish verrucous plaque on the right temple

The skin lesion was nail sized when first noticed 1½ years earlier, then gradually developed over the 8 months prior to presentation

The patient denied any associated fevers, weight loss, or night sweats

Physical examination revealed a slightly reddish verrucous mass with translucent papules and plaques on the right temple, about 5 cm in diameter, with surface hemorrhagic crust in the side adjacent to right eyebrow arch (Figure, A)

The lesion was nontender with no purulent discharge and no severe necrotic changes

There was no hepatosplenomegaly or superficial lymphadenopathy

Laboratory tests, including blood cell counts, urinalysis, and kidney and hepatic panels, revealed no abnormalities

A skin biopsy was performed and submitted for histopathologic analysis (Figure, B-D)

A, A slightly reddish verrucous mass with translucent papules and plaques on the right temple

B, The histological examination showed papillomatous epidermal hyperplasia, edema of dermal papillae, and neoplastic infiltration consisting of small round blue cells with alternating areas of dense and loose cellularity (hematoxylin-eosin)

C, Small, round blue cells were observed in the dermis with round, fusiform, or oval hyperchromatic nuclei, conspicuous nucleoli, and scant cytoplasm (hematoxylin-eosin)

D, Tumor cells were positive for MyoD1 (original magnification ×400)


Epstein-Barr virus–positive mucocutaneous ulcers

Crohn disease associated with pyostomatitis vegetans

A man in his 40s with a long-standing history of poorly controlled HIV (multiple treatment lapses, viral load of 22 871 copies/mL 8 months prior to presentation but now undetectable with use of highly active antiretroviral therapy, with a CD4 count of 40 cells/mm3) presented to the emergency department with nausea, abdominal pain, bloody stools, odynophagia, and recurrent oral and perianal ulcers and was found to have pancytopenia and septic shock

During admission in the intensive care unit, treatment with broad-spectrum oral antibiotics for presumed infection and oral fluconazole for thrush led to improvement in the patient’s medical condition, but the ulcers persisted

Dermatology was consulted for input on the cause of the ulcers, which had been ongoing for approximately 6 months prior

individual lesions lasted 1 to 3 weeks before self-resolving

Biopsy of an oral mucosal lesion performed 1 month earlier had shown nonspecific inflammation

Examination revealed exquisitely tender, ovoid, punched-out ulcerations of the inferior lip and scrapeable white plaques on the tongue (Figure, A), right buccal mucosa, and external anal mucosa (Figure, B)

A punch biopsy from 1 of the perianal ulcers was performed (Figure, C and D)

A, Healing oval punched-out ulceration of inferior lip and white plaques on tongue consistent with thrush

B, Two ovoid, punched-out ulcerations of the external anal mucosa

C, Hematoxylin-eosin staining of perianal lesion showed mixed infiltrate of histiocytes, neutrophils, lymphocytes, and eosinophils, with prominent admixed large and atypical cells at high power

D, Epstein-Barr virus–encoded RNA staining showed scattered positivity in some cells


A 50-year-old man was referred for ophthalmoplegia that developed immediately after an 8.5-hour robotic cystectomy for small cell cancer of the bladder

After recovering from anesthesia in the postanesthesia care unit, he experienced headache, facial numbness, inability to open both his eyes, and inability to move both eyes in any direction

He had a known enhancing central skull base lesion in sella turcica with compression of the optic chiasm and mild mass effect on the pons that was felt to be a pituitary macroadenoma (Figure)

His baseline examination before the cystectomy showed visual acuity of 20/20 OU with intact extraocular movements and unremarkable confrontational fields, although a mild bitemporal hemianopia was seen on automated visual fields

T1-weighted coronal magnetic resonance imaging (MRI) with contrast shows a 4.9 × 4.6 × 5.2-cm sellar/parasellar lobulated mass with homogenous enhancement before symptom onset (A), which became 5.3 × 5.1 × 5.3 cm and had loss of enhancement after symptom onset (B)

The mass caused superior displacement of the optic chiasm and had extension into the cavernous sinus bilaterally

The mass also extended posteriorly and deformed the anterior aspect of the pons (not shown)

After symptom onset, the mass was hyperintense on T2-weighted images (not shown)

During the bedside examination in the postanesthesia care unit, he was noted to have complete bilateral ptosis

His visual acuity was 20/20 OU with full visual fields on confrontational visual field testing

He had decreased sensation to light touch and pinprick in the V1/V2 distribution of the trigeminal nerve bilaterally

The right and left pupils were 5 mm and 6 mm in size, respectively, in both light and dark

There was no pupillary response to light or accommodation

Intraocular pressure was normal in both eyes

The patient was unable to move either eye in any direction on extraocular motility testing

Vestibulo-ocular reflex did not overcome the ophthalmoplegia

Magnetic resonance imaging (MRI) was obtained 12 hours after symptom onset redemonstrating the large sellar mass, which no longer had central enhancement (Figure)


Superior semicircular canal dehiscence syndrome

A 51-year-old woman presented with a 24-hour history of severe dizziness that she described as “room spinning,” right aural fullness, and a subjective right-sided hearing loss

She denied any medical history of vertigo, recent upper respiratory infection, head trauma, or prior otologic surgery

A physical examination revealed no abnormalities on otoscopic evaluation

however, a strongly positive right-sided Dix−Hallpike test was elicited with rotary nystagmus

a subsequent Epley maneuver was performed

The patient’s symptoms subjectively worsened during the following several days

Therefore, an audiogram test (Figure 1) and computed tomography (CT) of the temporal bone were performed

Preoperative audiogram reveals a right-sided low- to mid-frequency moderate conductive hearing loss and a bilateral high-frequency sensorineural hearing loss


An 18-year-old male presented to the primary care physician with a 4-month history of a slowly progressive painless lateral neck mass on the right side

The patient denied any history of dysphagia, dysphonia, drainage, previous neck surgery, trauma, or recent fever

There was no significant medical or family history of thyroid disease

On examination, the mass moved with swallowing

The patient had normal thyroid stimulating hormone and calcium levels and a parathyroid hormone (PTH) level of 63 pg/mL

Neck ultrasonography revealed a well-circumscribed anechoic lesion with a diameter of 5.9 cm in the right thyroid lobe without any internal solid nodularity or septation

A neck computed tomography (CT) scan revealed an intrathyroidal lesion extending inferiorly to 3 cm above the angle of Louis causing tracheal compression and displacement (Figure, A)

The patient underwent right hemithyroidectomy to relieve tracheal compression

A near-infrared autofluorescence (NIRAF) camera yielded a homogenously dim lesion (Figure, B)

Pathologic examination confirmed a normal right thyroid lobe, a normal right parathyroid gland, and a cystic lesion at the inferior pole with no evidence of malignant disease in the specimen

The tissue inside the lesion stained positive for PTH

A, A neck CT scan showing an intrathyroidal mass causing tracheal compression and is in close proximity to the right common carotid artery and right internal jugular vein

B, A NIRAF image of the surgical field showing right thyroid lobe, a right parathyroid gland, and a homogenously dim capsulated lesion of interest


A 53-year-old woman presented to the emergency department after awakening with right preauricular and right upper neck swelling

She had a history of coronary artery disease and prior myocardial infarction that was treated with warfarin

She denied any personal or family history of cancer

A physical examination revealed no erythema or tenderness of the parotid

Her cranial nerves were intact, specifically the right facial nerve and its branches

The oral mucosa was intact with no epithelial lesions

Urinalysis results revealed slight hematuria

Her international normalized ratio (INR) was 4.4. Contrast-enhanced computed tomography (CT) revealed an ill-defined infiltrative mass centered in the deep lobe of the right parotid with extension into surrounding soft tissue spaces (Figure, A)

A follow-up CT was performed 6 weeks later (Figure, B)

A, Axial contrast-enhanced computed tomography (CT) reveals an ill-defined, infiltrative lesion centered in the deep lobe of the right parotid gland


Lipodystrophia centrifugalis abdominalis infantilis

Subcutaneous fat necrosis of the newborn

A 2-year-old girl who was otherwise healthy presented with a 10-month history of asymptomatic skin depressions in her neck, nape, and submandibular regions

The lesion initially occurred as an erythema in the neck and progressed centrifugally to the nape and submandibular areas

Atrophy was observed in the center of the lesion, surrounded by an erythematous border, with underlying blood vessels being visible deep to the atrophy (Figure, A)

A, Atrophy in the center of the lesion, surrounded by an erythematous border, with underlying blood vessels deep to the atrophy

B, A biopsy specimen taken from the edge of the depressed lesion showing normal epidermis and dermis, no inflammatory cell infiltration within the dermis, and somewhat diminished subcutaneous fatty tissue (hematoxylin-eosin)

C, The same specimen at a greater magnification (hematoxylin-eosin)

D, Ultrasonographic findings showing markedly thinner subcutaneous fat of the skin (thickness of thinner region, 0.08 cm)

Cervical lymph nodes were palpable bilaterally

Tactile responses within the depressed as well as surrounding areas were normal

Other physical examinations were without abnormalities

Her medical and family histories were unremarkable

Her parents denied drug injection or trauma history

Results of routine laboratory tests, HIV test, serological test of antibodies for Borrelia, and antinuclear antibody test were within normal limits or negative

After obtaining informed consent from her parents, a biopsy specimen was taken from the edge of the depressed lesion for histological examination


A woman in her 40s presented with painful erythematous plaques on her trunk (Figure, A)

Skin lesions developed 2 months earlier and progressively spread

She reported abdominal discomfort, weight loss, and vaginal bleeding

A solid mass on her lower abdomen that had been palpable about 10 years prior had rapidly increased in size within the past few months

Physical examination revealed erythematous plaques on the right chest and flank without warmth

Punch biopsy of a lesion from the chest was sent for histopathological examination (Figure, B and C)

A, Erythematous plaques involving the right chest and flank at the initial presentation

B and C, Large atypical cells with clear or eosinophilic cytoplasm forming papillary and glandular architectures are present in the deep dermis

lymphovascular invasion was observed in the superficial and deep dermis (hematoxylin-eosin)

D, Contrast-enhanced computed tomographic scan of the abdomen and pelvis showing a large heterogeneous mass in the right rectus abdominis muscle (blue arrowhead) and cystic lesion in the left ovary (pink arrowhead)


Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes

A man aged 44 years was referred for progressive parkinsonism with poor levodopa response

His medical history comprised unexplained sensory hearing loss, hyperlipidemia, and osteoarthritis

At age 23 years, he had bilateral cataract surgery, and over the last 2 years, he developed worsening stiffness and walking difficulties with frequent falls

He reported features of rapid eye movement (REM) sleep behavior disorder, restless legs syndrome (RLS), visual daytime hallucinations, and rapid cognitive decline

He was given levodopa/benserazide (up to 200 mg/50 mg 4 times daily) without improvement on his Unified Parkinson Disease Rating Scale scores

Therefore, his dopaminergic medication was ceased

Family history was negative for movement or developmental disorders

His neurologic examination showed hypomimia, symmetrical bradykinesia, and rigidity of the extremities without tremors (Video)

There was full range of eye movements with large-amplitude saccadic intrusions on smooth pursuit in all directions

Neuropsychological assessment revealed significant abnormalities in cognitive functioning, affecting verbal and nonverbal cognitive skills (ie, speed of information processing, visuospatial skills, verbal and visual memory loss, executive skills)

Mini-Mental Status Examination score was 26 of 30 points

Laboratory and cerebral spinal fluid testing (ie, paraneoplastic antibodies Hu, Yo, Ri, CV2, Ma2, amphiphysin, glutamic acid decarboxylase, N-methyl-d-aspartic acid, LGI1, and Caspr2, 14-3-3 protein) were normal

Cranial magnetic resonance imaging (MRI) showed mild frontal and parietal atrophy

An electroencephalogram demonstrated slowing in keeping with moderate generalized encephalopathy

Total-body positron emission tomography (PET) using fluorine 18 (18F) fluorodeoxyglucose (FDG) as a tracer showed no FDG-avid lesions

however, a subsequent brain-specific FDG-PET/MRI revealed hypometabolism throughout the left cerebral hemisphere and crossed cerebellar diaschisis (Figure 1)

As the patient’s cognitive function and parkinsonism had deteriorated rapidly, a brain biopsy of the left frontal cortex was done

Immunohistochemistry showed scattered globular inclusions consistent with Lewy bodies

A, Axial fluorine 18 (18F) fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging shows reduced FDG uptake in the left frontal, temporal, and occipital lobes, as well as subtle hypometabolism in the left basal ganglia and left thalamus

B, Crossed cerebellar diaschisis as a result of contralateral supratentorial asymmetry is seen in the coronal plane


A 63-year-old man presented with a history of previous left superficial parotidectomy 10 years before for a Warthin tumor

His histology results showed a 5-cm Warthin tumor, with a focus of nodular oncocytic hyperplasia in the adjacent parenchyma and reactive intraparotid lymph nodes

He presented to the ear, nose, and throat clinic and complained of pain in the operative site of 6 months duration

This occurred intermittently once a week and lasted for a few minutes each time

It was associated with a small lump over the left neck, and the pain was worse on touch and head turning

On examination, there was a 3-mm nodularity palpated deep to the neck wound along the mid sternocleidomastoid muscle

His facial nerve function was otherwise normal, and sensation in the ear lobe was intact

A magnetic resonance imaging (MRI) scan was performed, and the results showed no focal lesion in either parotid glands or at the area of interest over the left sternocleidomastoid muscle

The patient was counseled for an excisional biopsy of the left neck nodule

The intraoperative photograph of the neck and the excised specimen is shown in Figure 1.Intraoperative photograph of the left lateral neck and the excised specimen

The black arrowhead indicates left neck lesion, the white arrowhead the external jugular vein


Metastatic progression: proceed with irradiation

Radiation recall myelopathy: continue steroids, discontinue doxorubicin

Radiation necrosis: continue steroids, add bevacizumab

Spinal cord infarct: underlying cause workup, supportive care

A woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy

In 2020, she relapsed in the liver and spine

In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension

From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin

Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation

Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1)

Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued

Lumbar puncture showed no malignant cells

Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion

Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks

A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead)

Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging

B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket)


A 51-year-old woman was referred for evaluation of headache, right-side jaw pain, paresthesia, and trismus

The patient reported that the facial and jaw symptoms had evolved gradually over the past 5 years and that multiple medical therapies had provided only minimal relief of pain

The patient’s medical history included anxiety, depression, emphysema, hyperlipidemia, migraines, and pseudoseizures

She denied prior head trauma and had no pertinent surgical history

On physical examination, the patient had normal body habitus and reported bilateral tenderness of the temporomandibular joints and paresthesia of the right trigeminal nerve in the V2 and V3 distribution with intact masseteric nerve and symmetric facial nerve

Audiometric evaluation showed normal hearing bilaterally

Magnetic resonance imaging (MRI) revealed a cystic mass in the right side of infratemporal fossa (ITF) that was nonenhancing on T1 sequencing with contrast and hyperintense on T2 (Figure 1A)


An otherwise healthy man in his 20s presented to the dermatology department with thickening and swelling of hands after immersion in water, accompanied by burning pruritus for 3 years

The lesions occurred as symmetrical white and transparent papules, white keratosis, excessive wrinkling on the dorsum of hands after water immersion and worsened in summer, and remitted in winter

He was treated with topical tretinoin ointment intermittently per a diagnosis of chronic eczema at the local clinic with little improvement

The lesions gradually aggravated, and the affected area dispersed to wrists and elbows during past the 1.5 years

He attributed these changes to the need for washing hands frequently in the period of the COVID-19 epidemic

He had no family history of similar skin problems and did not have a history of hyperhidrosis, cystic fibrosis, atopy, or trauma to the hands

Physical examination showed some changes emerged on both hands after water immersion contrasting with any other condition (Figure, A and B)

We took a skin biopsy specimen from white papules on the right hand, and representative histopathologic features are shown in the Figure, C and D

A and B, After immersing both hands in water for 10 mins, there were symmetrical white papules, white hyperkeratosis spots, and excessive wrinkling on the thenar eminence, wrist flexure of both hands, and dorsum of fingers and hands

C and D, A skin biopsy taken from white papules after water immersion for 10 mins showed hyperkeratosis, dilated eccrine ducts in the epidermis and dermis, and hyperplasia of the eccrine sweat glanda (hematoxylin-eosin)


COVID-19−associated acroischemia (thrombotic vasculopathy)

A man in his 40s with a medical history of factor VII deficiency hemophilia presented with acute hypoxic respiratory failure secondary to COVID-19. Following admission, he was intubated with prone positioning, and treatment with tocilizumab and dexamethasone was started

He was also found to have Staphylococcus hemolyticus bacteremia, and treatment with cefepime was initiated

Three days after admission, the patient developed erythema and edematous necrotic plaques of the bilateral ears (Figure, A and B)

The necrosis progressed during the following days, primarily involving the earlobe of the right ear and a large portion of the helix of the left ear

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


A 32-year-old woman was referred by her dentist to an oral surgeon for evaluation of persistent bilateral cheek swelling and mouth pain

Two months prior to presentation, the patient had awoken with bilateral linear “gashes” in her mandibular vestibule that were accompanied by bilateral buccal facial swellings

Since that day, the facial swelling remained unchanged, and although the mucosal lesions healed, the oral pain persisted

The patient’s medical history was noteworthy for depression, for which she was taking bupropion and fluoxetine, and prior alcohol use disorder, for which she was 3 years sober

She denied any smokeless tobacco use but endorsed daily electronic cigarette use

The extraoral examination detected bilateral buccal facial swelling of the lower facial third without associated trismus or lymphadenopathy (Figure, A)

Intraorally, bilateral scarring of the mandibular buccal vestibule was noted with focal areas of ulceration

Small aphthous ulcerations were also present on the right buccal mucosa (Figure, B)

An incisional biopsy of the right vestibular lesion was performed, and the specimen showed diffuse plasmalymphocytic infiltrates within the lamina propria

On higher magnification, noncaseating granulomatous inflammation was observed (Figure, C)

Staining for acid-fast bacilli was negative

Clinical and histologic features of a patient with oral ulceration and facial swelling

A, Clinical examination shows bilateral buccal swelling

B, Intraoral examination shows linear vestibular ulcerations with buccolabial aphthous ulcers

C, Hematoxylin-eosin stain of biopsy specimen of the vestibular ulcerated tissue shows noncaseating granulomatous inflammation (original magnification, ×100)


A 64-year-old man presented to the otolaryngology clinic with hoarseness of voice for 3 weeks associated with intermittent dry cough

He had no medical history or family history of cancer and was not a smoker

In the absence of other suggestive symptoms, as well as a normal physical examination that did not reveal any enlarged cervical lymph nodes or oropharyngeal or neck mass and an unremarkable nasoendoscopic examination, a diagnosis of laryngopharyngeal reflux was made

The patient was advised to try conservative management with dietary advice and proton pump inhibitors, and a clinical review was scheduled in 6 weeks

The patient only returned 3 months later when his hoarseness became worse

Repeat nasoendoscopy now showed left vocal cord palsy in the paramedian position

In the absence of a history of trauma and associated neurological or systemic symptoms, the main concern was an underlying tumor that impinged on the vagus nerve at various points or its recurrent branch

Results of a computed tomographic (CT) scan showed an ill-defined soft tissue mass, which was likely nodal disease, at the left supraclavicular (Figure 1), as well as the prevertebral and paravertebral region, which partially encased the left common carotid, left subclavian, and left vertebral arteries

This was along the expected course of the left recurrent laryngeal nerve and likely accounted for the left vocal cord palsy

However, the primary site of disease remained unclear

A fused positron emission tomography with CT (PET/CT) scan with fluorodeoxyglucose (FDG) tracer was then performed

Coronal view of the computed tomographic scan of the patient’s neck, including the left supraclavicular node (yellow arrowhead) and the left common carotid artery (black arrowhead)


A patient in their 50s presented with asymptomatic keratotic papules, first noticed on the left middle and ring fingers and left toe web spaces more than 10 years earlier

The papules progressed proximally over the subsequent decade to involve the forearm, arm, scapular area, ankle, shin, and thigh, but only affected the left side of the body

The patient’s face, palms, soles, and entire right side of the body were spared

The patient has a history of rosacea being treated with topical metronidazole gel for flares but was otherwise asymptomatic

The patient did not have any alopecia, night blindness, or dry eyes or skin

There was no history of any exposure to radiation or arsenic, nutritional deficiency, inflammatory skin disease, or malignant neoplasm

There was no family history of a similar condition

Examination revealed multiple, submillimeter, skin-colored to yellowish keratotic spiny projections over the aforementioned areas, only on the left side of the body (Figure, A and B)

Punch biopsy from a lesion showed orthokeratosis with parakeratotic columns emerging from an epidermal invagination

The epidermis was mildly acanthotic and with a normal granular layer

There was a sparse superficial perivascular lymphocytic infiltrate

Adjacent hair follicles were unaffected (Figure, C)

A, Numerous, submillimeter, skin-colored spiny projections over left foot web spaces were present

B, Close-up photograph over the left scapular area showed lesions of similar morphologic characteristics

C, Histologic results showed a parakeratotic column overlying a mildly acanthotic epidermis with a normal granular layer and keratinocyte maturation

A sparse superficial perivascular lymphocytic infiltrate was present (hematoxylin-eosin)


Nonbullous neutrophilic lupus erythematosus

Palisaded neutrophilic and granulomatous dermatitis

A teenaged boy presented to the outpatient clinic with a 1-year history of asymptomatic cutaneous lesions on the face that had progressively spread to the trunk and extremities

He also had asthenia and mild intermittent arthralgias

Physical examination revealed multiple millimetric erythematous to hyperpigmented monomorphic keratotic papules involving the head, trunk, and extremities

Symmetric annular brown erythematous plaques on the cheeks and temples were also seen

There were no vesicles or pustules (Figure, A and B)

Laboratory investigations revealed a normal white blood cell count, mild anemia (hemoglobin level, 11.3 g/dL

to convert to g/L, multiply by 10.0), hypocomplementemia, and moderate proteinuria

Autoimmune serology results showed raised levels of antinuclear antibodies (>1:160), anti-Smith antibodies, SSA/Ro antibodies, SSB/La antibodies, and anti-U1RNP antibodies

Anti-dsDNA antibodies, lupus anticoagulant, rheumatoid factor, IgG and IgM anticardiolipin antibody, and anti-β2 glycoprotein 1 antibody results were negative

A biopsy specimen obtained from an erythematous papule on the trunk and stained with hematoxylin-eosin is shown in the Figure, C and D

A, Millimetric erythematous to hyperpigmented monomorphic papules involving the trunk

B, Erythematous papules and symmetric annular brown erythematous plaques on the cheeks and temples

C and D, Punch biopsy results (hematoxylin-eosin)


A previously healthy 8-year-old boy presented to an outpatient clinic for further evaluation of a fluctuating right parotid mass that had been present for 3 years

The patient was not immunocompromised and denied any trauma to the area

In the 2 months prior, he had received multiple rounds of antibiotics from other health care professionals

Outside computed tomography of the face showed a 3-cm cystic structure in the superficial aspect of the right parotid gland with associated lymphadenopathy

An outside health care professional had attempted incision and drainage of the area with negative cultures

The patient presented to our clinic with an isolated right 2-cm fluctuant and tender mass overlying the parotid gland

There was no accompanying cervical lymphadenopathy, and facial nerve function remained fully intact

The patient denied fevers or systemic symptoms

Results of laboratory evaluation revealed normal complete blood cell count and erythrocyte sedimentation rate levels with negative infectious workup results for Epstein-Barr virus, cytomegalovirus, HIV, and tuberculosis (TB)

Magnetic resonance imaging showed a 1.9-cm peripherally enhancing, centrally necrotic lesion overlying the superficial right parotid gland with multiple subcentimeter necrotic lymph nodes within the right parotid gland (Figure 1)

The T1-weighted, contrast-enhanced, and fat-suppressed axial (A) and coronal (B) magnetic resonance imaging scans demonstrate the enhancing right-sided lesions overlying the distal aspect of the parotid and extending deep into the parotid parenchyma


Intravascular papillary endothelial hyperplasia

Pseudo-aneurysmal metastasis of melanoma

A woman in her 40s with a medical history of multiple sclerosis consulted for the sudden development of 2 adjacent nodules on the left arm

An excision with 1-cm margins of the left arm demonstrated desmoplastic melanoma with a Breslow thickness of 1 mm, and a left axillary sentinel lymph node biopsy (SLNB) had been performed 3 weeks before under general anesthesia

The patient was also being treated with subcutaneous interferon beta 1a for the multiple sclerosis

Physical examination revealed 2 indurated and mobile subcutaneous nodules on the anterior surface of the arm 5 cm distal to the surgical scar, the larger of them measuring 15 mm in diameter (Figure, A) and the smaller of them measuring 4 mm

When the arm was abducted to 90°, the patient denied pain but described a subtle tightness sensation on the arm

An excisional biopsy from the larger lesion was performed (Figure, B-D)

A, Clinical image showing indurated and mobile subcutaneous nodule on the anterior surface of the arm

Arrowheads highlight a subtle linear plaque adjacent to the nodule

B, Histopathologic image showing dilated and thrombosed lymphatic vessels in subcutaneous tissue with recanalization (hematoxylin-eosin)

C, Histopathologic image of partially thrombosed vessels

D, Immunohistochemistry finding was positive for D2-40 (podoplanin)


Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome

Chronic recurrent multifocal osteomyelitis

An otherwise healthy White man in his 60s presented with right jaw pain, trismus, and facial swelling

Symptoms started a year before, after receiving dental implants for new lower dentures

The patient noted right upper and lower eyelid swelling most notable in the morning but denied associated fevers, chills, vision changes, dysphagia, headaches, urinary symptoms, or rashes

Physical examination revealed a 3-cm round fluctuant area lateral to the right lateral canthus, mildly tender to palpation

No overlying warmth or erythema and no lymphadenopathy

The patient’s dentures appeared to be well fitting, but mouth opening was at approximately 50% of expected

Laboratory results included an erythrocyte sedimentation rate (ESR) of 20 mm/h, and normal complete blood cell count, comprehensive metabolic panel results,  and lactate levels

Findings on magnetic resonance imaging (MRI) (Figure, A) were most consistent with osteomyelitis of the mandible with inflammation of surrounding soft tissues

Right temporalis muscle biopsy results for Gram stain and culture, fungal stain and culture, and acid-fast bacillus stain were unremarkable

The patient’s symptoms did not improve after 7 weeks of empirical intravenous ertapenem followed by 8 weeks of oral cefuroxime and minocycline

Additional laboratory test results, including serum IgG4, SPEP, brucella AB, Q fever AB, RF, urinalysis, and microalbumin/creatinine levels, were all unremarkable

Results for p-ANCA and c-ANCA were negative

Repeated MRI findings showed progression of the inflammation and new involvement of the temporal lobe dura

Findings on positron emission tomographic computed tomography (PET-CT) scan showed nonspecific inflammatory changes to the bone, muscle, and brain meninges

A biopsy of the temporalis muscle, sphenoid wing, and dura was performed (Figure, B)

A, Magnetic resonance image (MRI) showing inflammation involving the right mandibular ramus and greater wing of sphenoid with overlying myositis involving masticator space

B, Histopathologic image of biopsy specimen (original magnification ×100) shows aggregates of multinucleated giant cells (arrowhead) associated with intima fibroplasia (hematoxylin-eosin)


Acute localized exanthematous pustulosis

A woman in her 20s was referred for evaluation of a 4-year history of recurrent pustular eruptions on her face, which initially presented while she was vacationing in Florida

She noted mild associated pruritus but denied burning sensation

The eruptions occurred intermittently, appearing as erythematous papules with prominent pustules, and were exacerbated by sunlight

On average, her lesions would resolve in 3 to 4 days with only mild background erythema by 5 to 7 days

Even mild sun exposure would induce the eruption, leading to frequent recurrences in summer months but long periods of remission in the fall through spring

Trials with various broad-spectrum sunscreens in combination with topical and oral antibiotics and topical ivermectin did not prevent flares or mitigate severity of eruptions when they occurred

She denied joint pain, fever, fatigue, changes in urine color, use of other topical or systemic medications, or presence of rashes elsewhere

She was otherwise healthy without other medical history

On physical examination, she was well appearing with numerous 1- to 2-mm monomorphous pustules over background erythema on the medial cheeks, chin, and nose (Figure, A)

No nail fold capillary changes or mucosal lesions were observed

Serum analysis results for antinuclear, anti-SSA/Ro, and anti-SSB/La antibodies were negative

A 4-mm punch biopsy was taken from a representative lesion along the right jawline (Figure, B-D)

A, Pustular eruption of the face characterized by monomorphous 1- to 2-mm pustules over background erythema on the cheeks, chin, and nose appearing less than 24 hours after sun exposure

Punch biopsy of skin demonstrated intrafollicular (B and C) and subcorneal (B and D) pustule formation (hematoxylin-eosin)