The patient denied any recent illness, travel, viral prodromal symptoms, headache, or skin lesions.

He reported no exposure to pets or animals.

A complete review of systems was otherwise negative.At presentation, his visual acuity was counting fingers OD at 1 ft and 20/100 OS, without an afferent papillary defect.

Slitlamp examination revealed fine inferior keratic precipitates bilaterally, with 3+ anterior chamber cells in the right eye and 2+ anterior chamber cells in the left eye.

Dilated ophthalmoscopic examination demonstrated numerous confluent creamy yellow lesions in the peripheral retina, with more discrete nummular lesions in the posterior pole in both eyes (Figure 1A).

No pigmented lesions were noted in the peripheral retina.

In addition, fine macular striae were present in both eyes, along with subtle optic nerve edema (Figure 1B).Case presentation.

A, Montage photograph of right eye shows lesions extending from the posterior pole to the retinal periphery.

B, Enlarged fundus photograph shows nummular macular lesions with mild retina striae and optic disc edema.


His perinatal history was notable for macrosomia, macrocephaly, left-sided failure on newborn hearing screen, and a congenital hypertrichotic patch of the left scalp.

During the first few months, he was observed to frequently tug at his left ear while displaying apparent discomfort and sleeping difficulties.

Swelling of the left lower part of the face was noted.

Of note, his length was in the 76th percentile for age, whereas his weight and head circumference were both greater than the 99th percentile for age.

A nontender, faint blue patch with subtle swelling and thick, coarse overlying hair was present on the left preauricular-temporal part of the scalp (Figure1A).

Subtle scattered tan patches were present on the trunk and extremities (Figure 1B).A, Blue patch of the left periauricular scalp.

B, Representative light tan patches with irregular borders on the back.

He was treated empirically with multiple antibiotics for presumed otitis media and subsequently had bilateral myringotomy tubes placed.

The dermatology department was consulted to consider treatment with propranolol.On examination, the patient was afebrile, intermittently irritable, but otherwise well appearing with stable vital signs.

The differential diagnosis included atypical infantile hemangioma, congenital hemangioma, or venous malformation.

When the left side of his face became more swollen, magnetic resonance imaging was performed at 8 months of age and revealed a left-sided parotid mass.

He was referred to our institution for further evaluation.Additional magnetic resonance imaging and magnetic resonance angiography of the brain and neck revealed an infiltrative vascular mass of the left parotid, skull base, and deeper facial structures with occlusion of the left internal jugular vein and extension into the external auditory canal.

Note the difference in the textural quality of the hair overlying the affected area, which the parents trimmed regularly to maintain a more uniform appearance.

A total of 15 similar lesions were noted on examination.


He had also noted an unintended 3-kg weight loss in the same duration, which he subjectively attributed to the stress of school.

He had experienced occasional blepharospasm and paresthesia in the ipsilateral eye and cheek.

Mild left-sided buccal weakness was noted.

Physical examination demonstrated a firm, immobile, irregular mass n the left parotid and approximately 3 × 3 cm in size.

Otherwise, his cranial nerve function was intact.

Fine needle aspiration demonstrated medium-size ovoid cells with minimal cytoplasm in crowded groups.

T2-weighted magnet resonance imaging following the initial computed tomographic (CT) scan demonstrated a poorly marginated mass that was 4 cm in maximum diameter abutting the left superficial parotid with involvement of the masseter (Figure, A).

Flow cytometry demonstrated phenotypically normal T-cell and B-cell populations.

Permanent histologic sections demonstrated nests of nonkeratinizing undifferentiated atypical epithelial cells intimately intermingled with a dense lymphoplasmacytic infiltrate (Figure, B).

Immunohistochemical analysis for cytokeratin AE1/3 was positive in the carcinoma cells (Figure, C).

In situ hybridization for Epstein-Barr virus–encoded small RNAs (EBERs) was positive (Figure, D).A, T2-weighted coronal magnetic resonance image of the face.

A parotidectomy with excision of the tumor was planned.

During the initial exposure of the tumor, an intraoperative frozen section demonstrated a cellular infiltrate, and a diagnosis of lymphoma could not be excluded.

Given the size and fixed nature of the tumor, the decision was made to stop resection on establishing a definitive diagnosis.

B, Hematoxylin-eosin, original magnification ×20.

C, Cytokeratin AE1/3 immunostain, original magnification ×20.

D, Epstein-Barr virus in situ hybridization, original magnification ×20.


It was associated with minimal pain and intermittently fluctuated in size.

Physical examination revealed a single 1.0 × 0.7-cm mass on the left mid helix that was firm, nontender, nonerythematous, and nonerosive.

The lesion was found to be superficially located in the dermis and filled with a soft white material that appeared as purple to blue crystals.Histopathologic images.

A, Specimen shows purple to blue crystals with cystic components.

B, Cystic structure with amorphous material and granulomatous reaction at the periphery with occasional foreign-body giant cells.

The resected specimen appeared cystic (Figure, A) with granulomatous reaction at the periphery (Figure, B).


Her mother had a full-term vaginal delivery without complications and a medical history that was notable only for gastroesophageal reflux disease treated with ranitidine.

The parents first noticed a pea-sized nodule in front of her left ear that was now 3 cm, soft, mobile, and nontender, without overlying skin changes or facial nerve impairment.

The infant was otherwise well without fevers, feeding well, and growing appropriately.

Ultrasonography showed a 2.2 × 3.0-cm solid nodule.

The patient was then referred for magnetic resonance imaging (MRI) with contrast, which showed a 2.9 × 1.6 × 2.3-cm mass with a well-defined capsule arising from within the superficial left parotid gland.

The mass was largely of fat signal intensity on both T1-weighted (Figure, A) and T2-weighted sequences with heterogeneous soft-tissue enhancement on T1 post-contrast (Figure, B).

The mass also suppressed relatively uniformly on short T1 inversion recovery (STIR) sequence (Figure, C).

The Figure, D, shows the mass in situ during surgical excision with facial nerve monitoring.Magnetic resonance images of a patient with a left preauricular mass of the superficial parotid lobe.


His medical history was significant for blunt trauma to the left maxilla region approximately 10 years prior to presentation.

His surgical history was noncontributory.

The pain was exacerbated by eating and prolonged talking.

He was initially seen at an emergency department, where his symptoms were unsuccessfully treated with antibiotics.

He stated that he had not experienced hoarseness, weight loss, fever, night sweats, parotid mass, or involuntary facial nerve spasms.

Physical examination demonstrated a well-developed man in no distress who spoke in a normal tone.

The external auditory canals and the tympanic membranes were intact and clear bilaterally.

The nasal cavity and the nasopharynx were normal.

The oral cavity and oropharynx revealed moist mucosa with no lesions or erythema.

Fiber-optic flexible nasopharyngoscopy and laryngoscopy revealed normal pharyngeal walls and pyriform sinuses.

The vocal folds were symmetric and mobile bilaterally.

Computed tomography (CT) of the neck demonstrated a ossified mass (arrowheads) in the left parapharyngeal space (Figure).


Her medical history was significant for a single episode of lower-extremity deep venous thrombosis during a prior hospitalization, complicated by multiple subsegmental pulmonary emboli.

At that time, she underwent inferior vena cava filter placement, followed by initiation of anticoagulation therapy.

She denied any substantial tobacco or alcohol use, and her family history was negative for venous thromboembolic disease.

Her initial physical examination had unremarkable results, with laboratory evaluation of prothrombin time significant for an international normalized ratio of 2.8.

Computed tomography (CT) of the chest revealed a large filling defect within the main pulmonary artery, flattening of the interventricular septum, and multiple pulmonary nodules.

Bedside transthoracic echocardiography demonstrated evidence of severely increased right ventricular systolic pressure, with moderate right ventricular dilation.

She was referred for urgent pharmacomechanical interventions, including catheter-directed embolectomy and thrombolysis, for a presumed diagnosis of submassive pulmonary embolism.

Following the procedure, she experienced no substantial clinical or radiographic improvement.


The immunoglobulin heavy chain (IgHV) gene mutational status was studied at this time and found to be unmutated, with somatic mutation rate of 0%.

Immunophenotyping was typical for CLL, showing expression of CD 5, CD 19, CD 23, CD 38, and dim κ light chain restriction.

Fluorescence in situ hybridization was positive for del (13)(q14) in 63% of her cells.

Lymphadenopathy was noticed during the initial physical examination and imaging studies.

Repeated cytogenetic tests showed complex karyotype with del(17)(p13.1) in 35% of her cells as a new abnormality.

Owing to progressive disease, she was started on therapy with fludarabine and rituximab (FR) 6 months later for 4 cycles, and clinical complete remission was achieved (bone marrow assessment was not done).

However, 2.5 years later she experienced disease relapse.

Owing to developed worsening fatigue and lymphadenopathy, she wanted to discuss treatment options.The patient is much less likely to respond and have a durable remission with chemoimmunotherapy.The patient is at lower risk of Richter transformation to diffuse large B-cell lymphoma.IgHV-mutated status is defined by more than a 2% sequence difference compared with the germline sequence and can change over the evolution of the disease.The patient is likely to have unfavorable cytogenetic features, positive ZAP-70 protein expression/promoter unmethylated status, and clonal evolution.


The patient did not have abdominal pain, nausea, vomiting, or a history of abdominal surgical procedures and did not use alcohol or tobacco.On physical examination, the patient’s abdomen was soft, nontender, and nondistended, with no palpable masses, and he did not appear jaundiced.

Results of laboratory testing demonstrated a carbohydrate antigen 19-9 level of less than 1 U/mL and a carcinoembryonic antigen level of 6.0 ng/mL (to convert to micrograms per liter, multiply by 1.0).

A duodenal mass was found using esophagogastroduodenoscopy and biopsies were taken.

To further evaluate the lesion, the patient underwent a computed tomographic scan of the abdomen and pelvis with contrast dye (Figure 1).A, Cross-sectional computed tomographic scan demonstrating a 4.7 × 2.9-cm, heterogeneous, solid mass involving the ampulla of Vater and the head of the pancreas.

A heterogeneous soft-tissue nodule can be seen (arrowhead).

The common bile duct (CBD), duct of Santorini (S), and duct of Wirsung (W) can all be identified traversing the mass compressing the duodenum (D).

B, Coronal computed tomographic scan demonstrating a polypoid pedunculated ampullary tumor extending into the duodenal lumen.


The patient had undergone laryngectomy with radiation, modified radical neck dissection, and a pectoralis major flap into the right-sided neck wound in 1998.

This operation was subsequently complicated by right carotid blowout requiring right-sided carotid artery ligation on November 5, 2005.

The patient initially presented to our hospital with a Glasgow Coma Scale score of 3.

Pertinent findings included an improved Glasgow Coma Scale score of 12 and left arm and leg hemiparesis.

The right upper arm was cool to the touch below the elbow, with cyanotic right fingers, and the right-sided radial and brachial pulses were absent.

The patient also presented with a pulsatile mass in the upper chest wall on the right side.

Findings from computed tomography of the head at the referring hospital showed acute right-hemispheric stroke with left-sided hemiparesis.On examination, the patient was afebrile, with a heart rate of 108 beats/min and blood pressure of 169/91 mm Hg with 100% oxygen saturation.

B, Selective angiogram of the innominate artery.

A confirmatory magnetic resonance image of the head is shown in Figure 1A, and a selective angiogram of the innominate artery is shown in Figure 1B.A, Magnetic resonance imaging of the head.


He was adherent to his antiretroviral therapy (ART) regimen (tenofovir/emtricitabine, raltegravir), with a recent CD4 lymphocyte count of 382 cells/mm3 (18%) and HIV RNA below the level of assay detection.

He reported taking atorvastatin for dyslipidemia and testosterone gel for hypogonadism.

He denied having jaundice, pruritus, abdominal pain, or other gastrointestinal symptoms and reported no alcohol intake or recent use of new medications.

Shortly thereafter he developed fever to 38.9°C and a macular rash that involved his palms and soles (Figure).

Initial evaluation was unremarkable except for new abnormalities in hepatic laboratory results (alanine aminotransferase, 217 U/L (3.6 μkat/L); aspartate aminotransferase, 149 U/L (2.5  μkat/L); alkaline phosphatase, 610 U/L (10.2 μkat/L); total bilirubin, 2.2 mg/dL (37.6 μmol/L); and albumin, 3.1 g/dL).

Test results for hepatitis A, B, and C were negative, as were results for anti–smooth muscle and antimitochondrial antibodies.

Results of a serum antinuclear antibody test were positive, with a titer of 1:640.

Liver biopsy demonstrated moderately active interface and lobular hepatitis with plasma cells and periportal cholestasis, findings suggestive of autoimmune hepatitis.

Physical examination was otherwise normal.Left, Pigmented, macular rash on palmar surface of left hand.

Right, Pigmented, macular rash on plantar surface of left foot.

The patient was started on prednisone and azathioprine.


He had no personal history of cancer, venous thrombosis, recent surgery, trauma, or admission to hospital.

His medications included fluoxetine, atorvastatin, vitamin D, magnesium, and niacin.On examination, his blood pressure was 164/82 mm Hg; pulse rate, 104/min; and respiratory rate, 18/min.

He described increased anxiety and a pleuritic chest pain that began a few hours prior to his ED visit, but no hemoptysis.

Oxygen saturation level was 96% in room air.

His lungs were clear to auscultation bilaterally.

No extra heart sounds or murmurs were noted.

Examination of his extremities were unremarkable with no leg symptoms or swelling or pain at calf palpation.

Laboratory test results showed a hemoglobin level of 160 g/dL (reference range, 14.0-17.5 g/dL), a platelet count of 129 × 103/μL (reference range, 150-350 × 103/μL), and a serum creatinine level of 1.26 mg/dL (reference range, 0.6-1.2 mg.dL) (111 μmol/L; reference range, 13-106 μmol/L).

The attending physician’s presumptive diagnosis was pulmonary embolism (PE).

His pretest clinical probability of PE according to the 2-level Wells score1 was likely (1.5 points for heart rate >100, and 3.0 for other diagnoses less likely than PE).

Therefore, D-dimer measurement was not performed.

His computed tomographic pulmonary angiography (CTPA) result is reported in the Table.Rule out PE and perform a work up for alternative diagnoses.Obtain a bilateral lower limb veins compression ultrasonography.Obtain a lung ventilation-perfusion (V̇/Q̇) scan or pulmonary angiogram.


He moved to Texas from the northeast a few months prior to his illness.

He denied any exposure to sexually transmitted infections or a history of genital lesions.

He reported that a fraction of the lesions had blistered earlier in the course of his illness.

The patient also described myalgias and fatigue over the preceding month.

On examination, the patient had erythematous papules on bilateral palms and soles as well as eroded vesicles on the palate (Figure, A and B).

A biopsy specimen of a papule on the left sole was obtained for immunohistochemical staining (Figure, C and D).

A serologic antibody test revealed an elevated coxsackievirus titer of 1:16 (reference range, <1:8; >1:32 indicative of infection).A, Patient’s soles at presentation.

C, Hematoxylin-eosin–stained section of the biopsy specimen (original magnification, ×20).

D, Immunohistochemistry stain of biopsy specimen (anti–Treponema pallidum polyclonal antibody immunostain; original magnification, ×20).


He stated that he did not have a history of poor wound healing or painful skin ulcers.

He had a history of type 2 diabetes and an allogenic bone marrow transplant 1 year prior for myelodysplastic syndrome complicated by pancytopenia and chronic graft-vs-host disease.

He reported subjective fevers, as well as increasing erythema and tenderness immediately around the right forearm lesion.

Physical examination revealed an isolated slightly erythematous, edematous plaque with a prominent central eschar on the right forearm (Figure, A).

An incisional biopsy at the edge of the eschar was performed, and a specimen was sent for pathological evaluation (Figure, B-D).A, Clinical photograph of a nonhealing ulcer with central eschar on the right forearm.

B, Necrotic debris and fat necrosis with saponification (hematoxylin-eosin, original magnification ×40).

C, Saponification and needle-like clefts within necrotic adipocytes (hematoxylin-eosin, original magnification ×200).

D, Numerous nonseptate, thick-walled fungal hyphae in the dermis (hematoxylin-eosin, original magnification ×400).


He reported that the lesions had begun 6 months previously and that they were most bothersome at the end of the day or when he was exposed to the cold and damp environment while out on his boat.

He applied ice to alleviate the burning, but this worsened the condition.

Histologic findings from biopsy specimens were also obtained by routine histologic analysis (Figure, B) and direct immunofluorescence examination (Figure, C).A, Clinically, the left ear demonstrates erythema, edema, and focal crusting.

B, Biopsy specimen from the left ear reveals thrombi within engorged blood vessels but fails to reveal significant inflammation of blood vessel walls (hematoxylin-eosin, original magnification ×200).

C, Biopsy specimen from the left ear under direct immunofluorescence and stained for anti-IgM highlights thrombi within vessels (original magnification ×200).

A heating pad provided some measure of relief, as did a brief course of systemic corticosteroids.Physical examination revealed that some papular lesions were associated with slight purpura and hemorrhagic crust (Figure, A).


The older brother had an extensive medical history, including dysphonia, oral mucosal lesions, alopecia, recurrent parotitis, vocal cord swelling, and recurrent vesicular eruptions on his face, trunk, and perineum.

Two skin biopsies were performed at 2 and 6 years of age, but the findings were considered unremarkable.Findings from ophthalmological examination of the older sibling showed confluent waxy nodular lesions extending from the lashes to the mucocutaneous junction on all 4 eyelids (Figure 1), impairing eyelid closure bilaterally.

There was no history of associated eyelid pain or bleeding.

Focal trichiasis was associated with superficial punctate keratitis.

Waxy nodular lesions (arrowheads) are present along the upper and lower eyelids of the patient’s left eye.The younger brother had similar, although less severe, ocular and systemic findings, including eyelid nodules, facial scarring, hyperkeratosis, oral mucosal lesions, and dysphonia.

There were no lesions on the conjunctiva or iris bilaterally.

The lenses were clear and no abnormalities were visualized using funduscopy.

Findings from the remainder of the ocular examination were unremarkable.Clinical appearance of eyelid margin nodules in the older brother.

He had seen several medical specialists and had been comprehensively evaluated for these concerns yet remained undiagnosed since birth.

The patients’ parents denied consanguinity.


Five days later, he developed painless horizontal diplopia and returned to the emergency department.

Alternate cover testing revealed a 30 prism diopters (∆) esotropia on primary gaze that increased to 45∆ on lateral gaze.

Additional evaluation included a repeated computed tomographic scan that showed no infectious involvement of the orbits or petromastoid complexes (Figure).

Findings from magnetic resonance imaging of the patient’s brain and orbits did not reveal intracranial lesions or extraocular muscle myositis.

Results of lumbar puncture and cerebrospinal fluid testing, including opening pressure, cell count, and glucose and protein levels, were in the reference range.

The erythrocyte sedimentation rate and C-reactive protein levels were also in the reference range.

Findings from neurologic examination did not reveal paresthesias, difficulty with gait, or abnormal tendon reflexes.A, A computed tomographic scan of the face and orbits in coronal sections demonstrating inflammatory changes in the maxillary and ethmoid sinuses without orbital involvement.

B, An axial computed tomographic scan does not show inflammatory involvement of the petrous apices (digitally processed image).

A indicates anterior.The patient was then referred for neuro-ophthalmic evaluation.

Positive findings were limited to a −2.5 bilateral abduction deficit.

His pupils, intraocular pressure, visual fields, and findings from external examination were normal.

Findings from anterior segment and dilated fundus examinations were also normal.

Because of this infection, he went to an emergency department and was prescribed antibiotics for presumed sinusitis.

Findings from a computed tomographic scan showed diffuse sinusitis that was procedurally drained by an otolaryngologist.

However, this treatment did not resolve the diplopia and he was subsequently referred to a neurologist.

His best-corrected visual acuity was 20/20 OU.


He had 3 episodes of right-sided ptosis, each of which occurred at 18 months, 3 years, and 5 years of age.

None of these episodes was preceded by fever.

During the last 2 episodes, he experienced severe right-sided headache lasting for 6 to 7 days before development of ptosis; he also experienced double vision.

There was no history of loss of consciousness, seizures, loss of vision or hearing, facial deviation, trouble swallowing, or weakness of the extremities in any of these episodes.

At the time of the clinic visit, the patient was asymptomatic and results of the neurologic examination were normal.

Results of brain magnetic resonance imaging performed at 18 months showed thickening of the cisternal part of the right third cranial nerve in the perimesencephalic cistern (Figure, B) with no parenchymal lesions.

Results of tests on cerebrospinal fluid collected via lumbar puncture were normal.A, The patient’s right-sided ptosis during the first episode at age 18 months.

After the first episode, with a presumed diagnosis of acute disseminated encephalomyelitis, he received intravenous pulse methylprednisolone sodium succinate, 30 mg/kg, for 3 days and recovered completely in 3 weeks.

The patient received intravenous immunoglobulin, 2 g/kg, for 2 days after the subsequent 2 episodes, with a presumed diagnosis of chronic inflammatory demyelinating neuropathy, and recovered fully.

There was history of migraine on the maternal side of the family.

There was no family history of autoimmune disorders or cerebral aneurysms.

The family provided a photograph of the patient’s face at the time of the first episode (Figure, A).

B, Results of brain magnetic resonance imaging showing thickening of the cisternal part of the right third cranial nerve in the perimesencephalic cistern (white arrowhead).


He had no nasal obstruction, nasal bleeding, or feeling of aural fullness.

Fibroscopic evaluation revealed a black nevus–like lesion over the left torus tubarius, without surface necrosis or signs of bleeding when touched.

Examination of the neck, nasal cavity, and larynx showed no remarkable findings.

The margins were clearly identified, and laser excision was performed smoothly with only minimal bleeding.

Microscopy revealed small mixed serous and mucinous glands, normal respiratory epithelia with marked oncocytic cell changes, and abundant eosinophilic granular cytoplasm arranged in a tubular and microcystic pattern (Figure, B).

Scattered brown pigments were noted in the cytoplasm of most oncocytic cells (Figure, C).

The brown pigment was negative for iron stain (Figure, D) and HMB45 stain, but positive for Fontana Masson stain.

Ki-67 staining revealed low proliferative index in the lesion.

Some lymphocytic infiltration of the stroma was also noted.A, Endoscopic view of the nasopharynx.

It was recommended that he receive laser excision of the lesion under endoscopy.

During the surgery, the superficial lesion was found to be confined to the left torus tubarius, without invasion of the surrounding structures (Figure, A, arrowhead).

The surgical specimen was a single black, soft-tissue fragment measuring 0.4 × 0.3 × 0.2 cm.

B, Hematoxylin-eosin, original magnification ×100.

C, Hematoxylin-eosin, original magnification ×200.

D, Iron stain, original magnification ×100.


There was no other significant medical or surgical history.

On physical examination, severe trismus was present with maximal interdental distance of 1 mm.

A maxillofacial computed tomographic (CT) examination was performed (Figure).

A-C, Noncontrast computed tomographic images.

A, Axial slice from an image of the facial bones.

B, Coronal slice from an enhanced scan of the facial bones.

C, Coronal image of the facial bones using a soft-tissue algorithm.

Asterisk indicates left medial pterygoid artery.

D, Three-dimensional reconstructed image of the facial bones.

Approximately 10 years after onset of symptoms, she was seen by an oral surgeon who attempted mandibular release via a cervical incision.

The patient was brought to the operating room for excision.

According to the patient, the surgeon was “unable to reach” the affected area, and the operation was unsuccessful.


His medical history was significant for previous nasal endoscopy with biopsy of nasal polyps 6 years previously, with a finding of benign nasal polyps by surgical pathology.

The patient had then been lost to follow-up.

He now complained of nasal drainage, severe headaches, and long-standing visual loss.

There had been no acute worsening of headaches or visual loss in the previous months.

Physical examination showed severe left proptosis, telecanthus, purulent nasal drainage, and nasal polypsis filling the bilateral nasal cavities.

Ophthalmology consultation revealed no evidence of restriction of gaze or papilledema, and a recommendation was made for endoscopic sinus surgery to remove the source of infection.

Computed tomographic imaging revealed extensive nasal polyposis, bony remodeling consistent with long-standing inflammation, and massive erosion of the bilateral skull base and orbits (Figure 1A).

Nasal polyps were present and had a typical appearance superficially (Figure 1B).

Tissue was sent for histologic evaluation.

The patient underwent endoscopic sinus surgery.

However, on deeper dissection, they became increasingly difficult to remove even with powered instrumentation, and an atypical, fibrotic endoscopic appearance was noted (Figure 1C).

Following this, the dissection was terminated.A, Coronal computed tomographic scan demonstrating extensive nasal polyposis and bony remodeling of bilateral skull base and orbits.

B, Rigid nasal endoscopy demonstrating superficial appearance consistent with nasal polyps.

C, Rigid nasal endoscopy following deeper dissection of area in question, demonstrating atypical fibrotic appearance.


She had a history of adenoid cystic carcinoma of the right submandibular gland and had undergone wide tumor excision and postoperative radiation therapy 20 years previously (60 Gy/30 fractions in 1993).

She also had a history of hyperthyroidism, which was under medical control (with propylthiouracil, 50 mg/d).

She had no family history of neurofibromatosis.

She stated that she did not have blurred vision, headache, seizures, or trismus.

A physical examination revealed a bulge in the floor of mouth, limitation of tongue movement, and no palpable lymphadenopathy.

Postgadolinium T1-weighted magnetic resonance imaging disclosed a 7.0 × 4.0 × 3.0-cm, well-defined enhancing mass in the right side of the floor of the mouth, extending posteriorly to the tongue base and medially across the midline (Figure, A).

The results of laboratory investigations were normal.

Transcervical removal of the tumor was performed.

She had no suspicious spots or nodules on the skin.

The surgical specimen was a 7.2 × 5.8 × 4.3-cm encapsulated tumor composed of a homogeneous, whitish, rubbery, and glistening content (Figure, B).

Microscopic examination demonstrated oval to eel-like spindle nuclei distributed in a background of generally wavy collagenous fibers and mucinous matrix (Figure, C).

The specimen showed sparse cellularity and no cellular atypia.

Neither mitotic figure nor tumor necrosis was found.

The specimen was immunoreactive for S-100 (Figure, D), focally positive for CD34, but nonreactive for smooth muscle actin.A, Axial T1-weighed gadolinium-enhanced magnetic resonance image of the neck.

B, Gross image of the surgical specimen.

Hematoxylin-eosin, original magnification ×200.


She presented with a 1-day history of sore throat, fever, chills, productive cough, globus sensation, dysphagia, and a change in her voice to the point it had become muffled and hoarse.

Throughout the day she noticed progressive worsening of her symptoms in addition to becoming dyspneic.

The patient tried using her albuterol inhaler, but this provided no relief of her symptoms.

The patient was afebrile at presentation, and there were no signs of infection.

On examination, there was mildly tender right cervical lymphadenopathy but no evidence of peritonsillar exudates.

Laryngoscopy demonstrated diffuse swelling of the supraglottic structures, particularly the epiglottis and left aryepiglottic fold, with a pale appearance of the mucosa (Figure, A).

In addition, a neck computed tomographic (CT) scan was obtained, which showed marked swelling of the supraglottic structures with areas of patchy enhancement and narrowing of the airway, as well as enlarged right cervical lymph nodes (Figure, B-D).

Owing to airway compromise, the patient was intubated and received intravenous steroids and ceftriaxone.

Subsequently, she underwent debulking surgery and carbon dioxide laser ablation of the left arytenoid.A, Laryngoscopic photograph.

B, Axial contrast-enhanced computed tomographic (CT) image through level of the aryepiglottic folds.

C, Axial contrast-enhanced CT through level of the epiglottis.


Her right eye had been enucleated 40 years before for choroidal melanoma, and she had been fitted with an ocular prosthesis (Figure 1).

Twenty-six years before presentation, she had received a diagnosis of moderately differentiated, estrogen and progesterone receptor–positive, invasive ductal carcinoma of the left breast (stage II, T2N1M0).

At that time, she underwent modified radical mastectomy, and 3 lymph nodes were positive for carcinoma.

She was then treated with 6 cycles of cyclophosphamide, doxorubicin hydrochloride, and fluorouracil, followed by daily tamoxifen citrate for nearly 5 years.

There was no evidence of recurrent disease after this treatment.

She denied pain, trauma, or changes in vision.

External examination revealed mild edema, erythema, and ptosis of the left upper lid (Figure 1).

Best-corrected visual acuity was 20/25, and left ocular motility was full.Photographs of the upper face show the ocular prosthesis (black arrowheads) and asymmetric enlargement, erythema, and ptosis of the left upper eyelid (white arrowheads).

Left cervical lymphadenopathy was present.

C, Right side.Recommend eyelid scrubs to the left eyelid for blepharitis.Refer for ophthalmologic examination and orbital computed tomography.Prescribe oral amoxicillin sodium–clavulanate potassium for infectious preseptal cellulitis.


Small free fluid within the pelvis and 2 large mesenteric masses were discovered adjacent to the liver, pancreas, and stomach (Figure 1).

During the trauma workup after a motor vehicle crash, 2 large peritoneal tumors were identified in her upper abdomen.

A, An exophilic mass in segment 3 of the liver invading the distal stomach.

B, A mass in the lessor sac attaching to the body of pancreas.

The arrowheads indicate 2 isolated tumors close to the liver, pancreas, and stomach.

The patient underwent exploratory laparotomy and en-bloc resection of the intraperitoneal tumors.Computed tomographic images.

An endoscopic ultrasound-guided fine-needle aspiration of the abdominal mass was obtained and the diagnosis was made.


Physical examination findings were notable for mild abdominal distension and tenderness without guarding or rebound sign.

He was afebrile and had normal vital signs.

Abdominal ultrasonography revealed a normal appendix.

Abdominal radiography (Figure 1A) revealed multiple polygonal calcified lesions in the pelvis and a nonobstructive bowel gas pattern.

Computed tomography (Figure 1B) revealed a 5.6 × 5.3 × 4.6-cm midline cystic pelvic mass adjacent to the anterior rectal wall that contained several irregularly shaped ossifications.

Laboratory study results were significant for normal levels of α-fetoprotein and chorionic gonadotropin and a white blood cell count of 19 700/μL (reference range, 5500-15 500/μL; to convert to ×109/L, multiply by 0.001).

The patient was taken to the operating room for an exploratory laparotomy.A, Supine abdominal radiograph reveals multiple polygonal calcified lesions in the pelvis (black arrowhead).

B, Computed tomographic sagittal image of the abdomen reveals a midline cystic pelvic mass that contains several irregularly shaped ossifications (white arrowhead).


His current medications include omeprazole and lorazepam.

The painful ulcers resolve within 2 weeks but recur on different mucosal surfaces.

He denies new medications, foods, or oral hygiene products and involvement of cutaneous or other mucosal surfaces.

Intraoral examination demonstrates an ulcer with a pseudomembrane and erythematous halo on the left lateral surface of the tongue (Figure).

He was previously prescribed anesthetic rinses and fluconazole, without benefit.

He is uncertain whether he has a family history of oral ulcers.


His resting oxygen saturation was 94%, declining to 86% after walking for 60 meters.

A pulmonary examination revealed inspiratory crackles at the bases and diminished breath sounds at the apices.

A cardiovascular examination showed normal results, without peripheral edema.

A chest x-ray showed hyperinflation and increased interstitial markings.


She reported a 6.3-kg weight loss, tremor, diaphoresis, and palpitations over the course of 2 months.

Physical examination revealed tachycardia, tongue and hand tremors, no exophthalmos, a 2-fold diffusely enlarged thyroid gland, and brisk reflexes.Laboratory results showed a thyroid-stimulating hormone (TSH) level of 0.005 mIU/L (normal, 0.45-4.50 mIU/L), free thyroxine level greater than 7.77 ng/dL (100.01 pmol/L) (normal, 0.82-1.77 ng/dL [10.55-22.78 pmol/L]), free triiodothyronine level of 30.6 pg/mL (47.1 pmol/L) (normal, 2.0-3.9 pg/mL [3.08-6.01 pmol/L]), and thyroid-stimulating immunoglobulin index of 5.5 (normal, <1.3).

Note the increased uptake in the thymus indicating increased metabolic activity.

The patient began treatment with methimazole, 20 mg twice daily.A noncontrast computed tomography (CT) scan of the chest (Figure, left) and an 18F-fludeoxyglucose–positron emission tomography (FDG-PET)/CT scan (Figure, right) were completed to evaluate her dyspnea.Left, Noncontrast computed tomography (CT) scan of the upper chest shown in cross section.

Right, 18F-fludeoxyglucose-positron emission tomography/CT scan of the whole body.


The parents reported occasional erosions and crusting that resolved with topical mupirocin, 2%, cream, but with no continued growth.

Physical examination revealed a firm, exophytic, and flat-topped nodule, with superficial erosion and crust on the right parietal side of the scalp measuring 3 cm in diameter and 1 cm thick (Figure, A).

Cerebral and abdominal ultrasonography revealed no evidence of visceral involvement.

A 4-mm punch skin biopsy was performed, and tissue was sent for pathologic analysis (Figure, B and C).

The lesion regressed without treatment over a period of 8 months.

Close follow-up every 3 months was scheduled, and complete involution of the tumor was noted at 10 months of age (Figure, D).A, Nodular lesion on the right parietal scalp at initial presentation.

B, Histopathologic image of a biopsy specimen (hematoxylin-eosin, original magnification ×200).

C, Immunohistochemical image of a biopsy specimen (actin stain, original magnification ×100).

D, Complete regression of tumor at 10 months of age.


His medical history included a heart transplant for ischemic cardiomyopathy in his early 50s, and he was receiving long-term immunosuppressive therapy with cyclosporine, mycophenolate mofetil, and deflazacort.

He had received a diagnosis of plasmablastic lymphoma 6 months earlier and was being treated with polychemotherapy (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone [CHOP] regimen).Physical examination revealed a well-demarcated 5 × 5-cm round ulcer with raised border, surrounding erythema, and a base with purulent debris (Figure 1A).

A punch biopsy specimen from the edge of the ulcer was obtained and sent for histopathologic evaluation (Figure 1B).A, Cutaneous ulcer on the patient’s left leg with a raised erythematous border and a base with purulent debris.

B, Diagnostic biopsy shows enlarged endothelial cells with intranuclear inclusions surrounded by a clear halo, and some cytoplasmic inclusions.

Similar findings are detected in some macrophages (hematoxylin-eosin, original magnification ×400).

A superficial swab culture sample grew P aeruginosa.


She was initially admitted for altered mental status in the setting of an unwitnessed fall and was noted to have “bruises” on her lower extremities.

Dermatology was consulted after the development of new lesions on her nose, ear, and the extensor surfaces of her arms bilaterally.

Admission laboratory test results showed hypercalcemia (calcium level, 11.5 mg/dL) and acute kidney injury (creatinine level, 5.6 mg/dL).On physical examination, the patient’s nose, bilateral cheeks, forehead, and left ear (helical rim and antihelix [Figure, A]) were found to have dusky purpuric macules and patches.

Her knees, feet, and the extensor surfaces of her arms had reticulated purpuric macules and patches.

There was skin sloughing off the lesions on her elbows (Figure, B) and knees.

Four-millimeter punch biopsy specimens were taken from the left ear, right arm, and forehead (Figure, C and D).Clinical and histopathologic images from a case of acral retiform purpura.

A, Dusky purpuric macules on the helical rim and antihelix of the ear.

B, Reticulated purpuric patches with focal areas of necrosis on the extensor surfaces of the arms.

C and D, In specimens from the forehead, multifocal intravascular fibrinoid material consisting of a mix of cryoglobulins and fibrin thrombi with relatively little vessel wall inflammation was seen (hematoxylin-eosin, original magnification ×4 [C] and ×10 [D]).


Her medical history was significant for Waldenström macroglobulinemia (WM), and recent laboratory test results showed active WM with bone marrow involvement.

Results of the eye examination showed best-corrected visual acuity of 20/20 OD and 20/40 OS.

Anterior segment examination results showed a relatively shallow anterior chamber in the left eye.

Results of a dilated fundus examination showed a choroidal detachment in the left eye extending circumferentially from the 2-o’clock to the 5-o’clock position and extending posteriorly up to 3 to 4 disc diameters temporal to the macula (Figure, A).

There was vitreous hemorrhage with a poor view of the posterior pole (Figure, A).A, Ultra-widefield fundus photograph of the left eye showing posterior vitreous hemorrhage (white arrowhead) and a choroidal detachment temporally (yellow arrowheads).

B, Anterior segment spectral domain optical coherence tomography of the left eye showing the narrow angle from suprachoroidal effusion.Results of fundus fluorescein angiography of the left eye showed hypofluorescence corresponding to vitreous hemorrhage.

Tortuosity of the temporal vessels was prominent, with diffuse peripheral vascular leakage temporally.

Choroidal folds were observed 3 disc diameters temporal to the macula, extending to the periphery.

Peripheral capillary nonperfusion, vascular reorganization, neovascularization, and choroidal detachment were noted.Anterior segment spectral domain optical coherence tomographic results showed a shallow anterior chamber and 360° narrow angles in the left eye (Figure, B).


Eight years earlier, he had sustained a fireworks injury to his right eye followed by globe repair and enucleation within 3 days.

He had no other significant medical or ocular history.His right eye’s external appearance was normal, and the prosthesis fit well.

He described a history of initially intermittent (lasting hours to days) then constant (for the past 6-8 weeks) central and peripheral blurred vision in the left eye.

He reported no headaches, numbness, weakness, eye pain, hearing loss, or other neurologic issues.

Uncorrected visual acuity was Snellen 20/70−1 OS (no improvement with pinhole); intraocular pressure was 13 mm Hg.

Slitlamp examination results of the anterior segment were normal.

Ophthalmoscopy demonstrated 1+ cells in the anterior vitreous and a serous retinal detachment involving the macula and extending from the 2-o’clock to the 10-o’clock position with pigmentary and atrophic changes in the macula.

No breaks were noted on scleral depressed examination.

Fluorescein angiography showed multifocal areas of early hyperfluorescence with late leakage and diffuse retinal vascular leakage predominantly in the inferior retina (Figure 1A).

Optical coherence tomography (OCT) showed a thickened choroid and subretinal fluid throughout the macula (Figure 1B).

Enhanced depth imaging showed an enlarged choroid throughout the posterior pole, and ultrasonography confirmed extensive inferior retinal detachment (not shown).A, Fluorescein late-phase angiogram shows multifocal areas of posterior choroidal leakage and diffuse retinal vascular leakage in the inferior (detached) retina.

B, Optical coherence tomographic en face infrared image (left) and vertical line scan (right) through the fovea show subretinal fluid extending inferiorly with hyperreflective subretinal deposits.


She first became aware of her increased abdominal girth when friends at school asked whether she was pregnant.

She had noticed her clothing becoming tighter over recent weeks.

She had not experienced abdominal pain or other symptoms, and there was no history of trauma.

Physical examination revealed a firm, distended abdomen without bowel sounds.

Results of laboratory studies, including a complete blood cell count, basic metabolic panel, and liver function tests, were within normal limits.

An abdominal radiograph showed a paucity of bowel gas markings (Figure 1A).

A subsequent computed tomographic scan of the abdomen and pelvis revealed a 30-cm retroperitoneal cystic structure causing significant mass effect on the abdominal viscera, displacing the right kidney superiorly under the liver causing moderate hydronephrosis (Figure 1B).A, Abdominal radiograph showing a paucity of bowel gas markings.


There was no history of immunocompromise or foreign travel.

He had no clinically significant improvement in symptoms despite multiple courses of oral antibiotics.

Over the previous 2 months, he had developed progressive right-sided facial swelling, pain, blurred vision, and a 6.8-kg weight loss.

The patient’s examination was notable for right-sided facial and periorbital swelling, severe pain, and limitation of lateral gaze of the right eye.

A computed tomographic (CT) scan at an outside hospital showed complete opacification and an invasive process centered in the right maxillary sinus.

A repeated CT scan was completed (Figure).A-C, Computed tomographic images.

A, Coronal view of the paranasal sinuses in bone windows showing near-complete opacification of the right maxillary sinus with soft-tissue thickening, a calcific focus, and associated erosion of the bony walls.

B, Coronal view of the paranasal sinuses in soft-tissue windows.

C, Axial view of the paranasal sinuses in bone windows.

The patient was started on treatment with intravenous antifungal and broad-spectrum antibiotics, and he was transferred for further evaluation and management.

An endoscopic maxillary antrostomy and biopsy showed inflammatory changes and numerous fungal organisms consistent with Aspergillus species.


He was a tour guide, a profession that requires speech.

Because he experienced voice fatigue frequently, he visited a hospital for professional assistance.

Fibroscopy showed a well-circumscribed mass of approximately 3 × 3 × 2 cm, with a smooth surface, located at the laryngeal side of the left aryepiglottic fold and covering nearby structures, such as the bilateral vocal cords and left pyriform sinus (Figure 1, A).

It was composed of adipocytes of various sizes in a fibromyxoid background.

In the fibromyxoid areas, atypical spindle cells with hyperchromatic and enlarged nuclei were present, as well as scattered lipoblasts without areas of dedifferentiation (Figure 1, B).

Immunohistochemically, the tumor exhibited substantial nuclear staining for both CDK4 and MDM2 and stains (Figure 1, C).

A biopsy was performed, and the patient visited our institution (Taipei Veterans General Hospital) for further intervention.

The laryngeal mass was removed through transoral laser microsurgery.

The tumor was at the submucosa with focally ulcerated overlying epithelium.


A magnetic resonance imaging (MRI) study of the brain and cervical spine obtained to evaluate for demyelinating disease demonstrated a normal brain and cervical cord, but an incidental T2 hyperintense lesion within the right pterygoid muscles was noted.

A dedicated fifth cranial nerve MRI examination performed to better characterize the lesion revealed a well-defined, lobulated T2 hyperintense lesion within the right pterygoid muscles without mass effect, adjacent edema, or other invasive features (Figure, A).

There was a small focus of signal void on the T2-weighted images, suspicious for tiny calcification.

On gadolinium administration, there was sequential increased enhancement on the postcontrast T1-weighted axial images (Figure, B and C).

Further imaging workup with single-proton emission computed tomography-CT (SPECT-CT) using technetium Tc 99m–labeled red blood cells (RBCs) demonstrated increased tracer pooling within the lesion (Figure, D).A-C, Magnetic resonance imaging studies (axial views) of a teenager with an incidental masticator space lesion.

D, Single-proton emission computed tomographic image (axial view) using technetium Tc 99m–labeled red blood cells demonstrating increased tracer pooling within the lesion.

The rest of the clinical history and neurological examination was unremarkable.

On further consultation with the otolaryngology department, the patient stated that she had not experienced any sensation of a mass, and results from a focused otolaryngologic examination were also normal.


The patient continued to experience dysphagia, globus, and a choking sensation when coughing.

A right pyriform sinus mass was seen on fiber-optic laryngoscopy.

Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the finding of a hypopharyngeal mass.

An MRI scan obtained 1 month postoperatively demonstrated a persistent mass or swelling.

On fiber-optic examination, right pyriform sinus fullness was visualized.

An interval MRI demonstrated a 2-cm soft-tissue mass in the cervical esophagus (Figure, A).

Using rigid suspension laryngoscopy, we identified a large, smooth-walled ovoid mass herniating from the cervical esophageal opening.

A mucosal stalk anchored the mass to the lateral pyriform sinus wall.

The tumor was well-defined, solitary, tan-red, and composed of sheets of large, round-to-polygonal cells with marked eosinophilic, granular cytoplasm (Figure, B).

Most tumor cells exhibited peripherally or centrally located vesicular nuclei with prominent nucleoli.

Peripheral intracellular vacuoles indenting the cytoplasm were seen, which represent a processing artifact due to intracellular glycogen loss (Figure, C).A, Axial contrast-enhanced spoiled gradient magnetic resonance image.

The patient underwent an endoscopic laser excision.

Endoscopic snare cautery was used to excise the mass.

Immunohistochemical stains revealed tumor cells that were focally positive for myogenin and diffusely positive for desmin and myoglobin.

During operative laryngoscopy, no mass was identified.

B, Hematoxylin-eosin, original magnification ×20.

C, Hematoxylin-eosin, original magnification ×40.


He reported a 44–pack-year smoking history and remote alcohol abuse.

On physical examination he had a 10-cm right-sided facial mass extending under the auricle from the mastoid to the zygoma and inferiorly to the angle of the mandible.

The mass was firm, mildly tender, but without overlying skin changes or cervical lymphadenopathy.

Computed tomographic (CT) and magnetic resonance imaging (MRI) scans showed maxillary sinus compression with partial destruction of the zygomatic arch, mandible, and orbital floor (Figure, A and B).

The mass extended into the right pterygopalatine fossa, disrupting the floor of the middle cranial fossa and widening the foramen ovale.

A core biopsy specimen revealed spindle cell proliferation with abundant collagen that stained focally with CD34, smooth muscle actin, and Bcl-2.

The Figure, C, shows a gross pathologic image after excision of the mass.Histopathologic findings demonstrated spindle cells arranged in long fascicles with abundant interstitial collagen (Figure, D).

Tumor cells exhibited central, oval nuclei, and moderate amounts of pale-pink cytoplasm with indistinct borders.

There was no increased or atypical mitosis, necrosis, or vascular invasion.

Immunostaining demonstrated strong nuclear staining for β-catenin antibody and was positive for smooth muscle actin antibody, while stains for desmin, pan-keratin, S-100, CD34, and Bcl-2 antibodies were negative.

The Ki67 index was less than 1%.A, Coronal computed tomographic scan of the mass.

B, Postcontrast axial T1-weighted magnetic resonance image.

D, Histopathologic image of excised tissue (hematoxylin-eosin, original magnification ×40).


He had no notable medical or surgical history.

He denied fevers, dizziness, or recent trauma.

He was afebrile with normal vital signs.

He was well nourished, alert, and in no acute distress.

Results of an abdominal examination revealed normoactive bowel sounds, no distention, but tenderness on the left side.

Computed tomography (CT) of the abdomen and pelvis with intravenous contrast revealed a 12 × 13 × 11-cm left-sided adrenal mass (Figure 1).

Results of laboratory examination revealed plasma levels of metanephrines, normetanephrines, cortisol, renin, and aldosterone within reference limits.


The patient had a history of multiple episodes of hematemesis attributed to alcoholic gastritis; however, at this admission, he indicated he had not experienced vomiting.

He described the pain as a sharp, left upper-quadrant pain migrating to the epigastric region for 24 hours.

He admitted to early satiety over the past few months.

Physical examination revealed a soft nondistended abdomen, but it was very tender in the epigastric region without rebound or guarding.

Elevated results of liver function tests, as well as amylase and lipase levels consistent with alcoholic pancreatitis, were noted.

Magnetic resonance imaging, computed tomographic imaging, and upper endoscopy findings are shown in Figure 1.

A, Computed tomographic scan and magnetic resonance imaging (inset) demonstrating a heterogeneous mass lesion in the fundus and body of the stomach.

B, Upper endoscopic image directly visualizing the mass intruding into the lumen of the stomach.

Biopsy results of the mass were reported as normal gastric mucosa.Diagnostic imaging.


Her medications included bimatoprost, atenolol, atorvastatin, and clopidogrel.A, Upper facial profile showed iris heterochromia and eyelash hypertrichosis.

Her irises were also different colors: the right iris was brown, and the left was hazel (green-brown) (Figure 1).

The patient had noticed a gradual darkening of her right iris and lengthening of her eyelashes during the last year but denied visual changes or foreign-body sensation.

B, Right eyelid and eye showed thick, long eyelashes with brown iris.

Her medical history included a cerebrovascular accident, hypercholesterolemia, and hypertension and a remote history of breast cancer treated with lumpectomy and radiation therapy.


His medical history includes a 5-pack-year smoking history but no illicit drug or alcohol use, promiscuous sexual activity, or recent travel outside of the United States.

He had tattoos placed 3 years before presentation and noticed skin lesions confined to his tattoos 1 year ago.

Vital signs revealed temperature, 36.7°C; heart rate, 76/min; respiratory rate, 17/min; blood pressure, 128/82 mm Hg; and oxygen saturation, 99% on room air.

Examination revealed a well-appearing patient with clear lungs bilaterally and numerous scattered red to violaceous macules and papules along his tattoos (Figure 1, left).

Complete blood cell count showed hemoglobin level, 11.3 g/dL; white blood cell count, 4.5 ×103/mm3; and platelet count, 189 ×103/mm3.

Results of a complete metabolic panel were normal except for elevated levels of blood urea nitrogen (33 mg/dL), creatinine (3.0 mg/dL), and ionized calcium (6.7 mg/dL).

Urinalysis showed gross hematuria, with 10 to 20 red blood cells per high-power field and 0 to 5 white blood cells per high-power field.

Chest radiography showed bilateral hilar lymphadenopathy without consolidations, nodules, or cavitary lesions (Figure 1, right).

Computed tomography of the abdomen showed right-sided hydronephrosis with an obstructive stone.Left, Numerous scattered red to violaceous macules and papules along patient’s tattoos.


His other medications include metformin and amlodipine.

He is married, has 3 children, and reports a sedentary lifestyle.

He has normal libido but experiences occasional erectile dysfunction, which is being successfully managed with a phosphodiesterase-5 inhibitor.

His morning total testosterone level (measured by mass spectrometry) was 279 ng/dL (normal reference range for this laboratory, 300-900 ng/dL), prompting his referral.

On physical examination, his body mass index was 34.7 (calculated as weight in kilograms divided by height in meters squared).He was not cushingoid and his visual fields were normal.

Acanthosis nigricans was noticed on the neck.

He recently heard on the radio that his symptoms might be due to “low T.” One month ago, he saw his primary care physician and requested measurement of his testosterone level.

Relevant laboratory tests, including repeat morning total testosterone assessed in an endocrinology clinic, are reported in Table.Further evaluation is required; repeat total testosterone measurement using an immunoassay.The patient has androgen deficiency; no further evaluation is required.


The lesions appeared while she was taking levofloxacin; however, on admission the antibiotic therapy was continued.

On physical examination, the patient had localized retiform purpura on the right earlobe and bilateral helices that were tender to palpation (Figure, A).

Initially, the left ear developed very painful lesions that resembled bruises.

Subsequently, her right ear became involved and developed similar lesions, but she denied any other areas being affected.

Findings from laboratory tests, including a hypercoagulability workup, complete blood cell count, comprehensive metabolic panel, urinalysis, cryoglobulins, cytoplasmic antineutrophil cytoplasmic antibodies, and complement levels were all unremarkable.

However, the test result for perinuclear antineutrophil cytoplasmic antibodies (p-ANCAs) was positive.

A punch biopsy for histopathologic examination was performed (Figure, B and C).A, Retiform purpura on the right helix.

B, Abundant red blood cell extravasation and fibrin thrombi (hematoxylin-eosin, original magnification ×10).

C, Leukocytoclastic vasculitis and fibrin thrombi (hematoxylin-eosin, original magnification ×40).

She had recently been treated for a urinary tract infection with trimethoprim-sulfamethoxazole and levofloxacin.

Her other medications included propylthiouracil, amlodipine besylate, pravastatin sodium, solifenacin succinate, and gabapentin.


The patient had no notable recent medical illnesses, illicit drug use, or new medications.

The lesions began on the bilateral lower legs and spread to the thighs, buttocks, hands, forearms, and proximal arms.

Review of systems was notable for episodic muscle cramping and numbness involving the lower extremities.

Physical examination revealed numerous stellate red-brown patches without induration, scale, or ulceration (Figure, A).

An incisional wedge biopsy was performed on a lesion from the lower extremity, and the specimen was stained with hematoxylin-eosin (Figure, B and C).A, Stellate red-brown patches on the patient's right lower extremity.

B, Specimen from a lower-extremity lesion (original magnification ×10).

C, Same specimen as shown in panel B but at higher magnification (×20).


There was no personal or family history of malignancy.

The patient noted that the lesion had slowly enlarged over the preceding 5 years from when he first noticed it as a small bump.

He denied a history of trauma prior to its development.

The patient denied any spontaneous bleeding, crusting, or itching, and he was primarily concerned about its growth.

On physical examination, the patient’s left frontal scalp revealed a mobile 1.2-cm mammillated, soft, brown plaque with protruding terminal hairs (Figure, A).

The remainder of the patient’s skin examination revealed no other concerning lesions.

Dermoscopically, the lesion was characterized by irregular pigment globules, milialike cysts, and focal arborizing vessels (Figure, B).

A shave biopsy was performed for histopathologic examination (Figure, C and D).


Her family history revealed that her father was alive and well at approximately age 90 years, and her mother had died at approximately age 80 years with a history of neuropsychiatric disease and gait change.

There was no history of tobacco, excess alcohol, or recreational drug use.

Her family noted that her first symptoms appeared at age 55, with subtle gait dysfunction and then evident imbalance.

By age 57, there was concern over progressive withdrawal from social interchange, personality changes, and impairment of judgment.

By age 59, she was experiencing bouts of inappropriate out-of-context laughter and tears, inattention, daytime fatigue, and frank memory impairment.

She developed visuoperceptive changes and difficulties using the television remote control device.

She developed obsessive checking behaviors.

She started having swallowing difficulties but nonetheless had unexpected weight gain.

She developed progressively worse motoric problems, with stiffness, abnormal posturing, a left toe pointing upward, and falls.

General examination findings were noncontributory.

Neurological examination revealed abnormal mental status, with full orientation and good registration, concentration, digit span, and language function but with poor calculations and constructions (Figure 1A) and decreased short-term memory.

Nonetheless, her Mini-Mental State Examination score was 28 of 30.

Cranial nerve examination revealed some decreased upgaze and mild facial masking.

Motor examination revealed rigidity and dystonic posturing in the left arm and leg, imbalance on gait, decreased arm swing, and retropulsion but no significant tremor.

Sensory examination findings were normal.

Reflex examination showed mild left hyperreflexia with an upgoing left toe.

Blood and urine laboratory study results were within normal limits.

Cerebrospinal fluid was unremarkable except for biomarkers that showed a mid-range β-amyloid 42 level of 530 pg/mL and normal total tau and phosphotau levels of 182 and 25 pg/mL, respectively.

Magnetic resonance imaging of the brain was reported as unremarkable (Figure 1B).

Positron emission tomography (PET) imaging of the brain revealed right frontotemporoparietal hypometabolism with some sensorimotor sparing (Figure 1C), which was neither typical nor atypical of Alzheimer disease.

Electroencephalography showed left frontotemporal sharp waves and slowing.

She had continued progressive decline in cognition and motor function and died almost mute and bedridden in her early 60s after a 9-year course of illness.A, Patient testing.

Constructional apraxia is seen in her copying of the top row template figures, with preserved writing in script.

B, Brain magnetic resonance imaging at the time of presentation.

The top panel shows selected sagittal T1-weighted images, and the bottom panel shows selected axial T1-weighted images.

C, Brain positron emission tomography using fluorodeoxyglucose F 18 tracer in the axial plane.

Frontal and temporal hypometabolism is seen, more marked on the right, with some apparent sensorimotor sparing bilaterally.


At presentation, she reported occasional photopsias, floaters, and blurred vision but no pain.

Visual acuity was 20/25−2 OD and 20/40 OS.

Low internal reflectivity and a mass measuring 8.4 mm in height by 12.9 mm across the base (Figure, A and B) were revealed on B-scan ultrasonography.

A complete blood cell count and results of liver function tests were within reference limits; a computed tomographic scan of the abdomen, chest, brain, and orbits showed no evidence of metastasis.A and B, B-scan ultrasonography of the left eye.

A, Original scan shows a mass with a height of 8.4 mm and a base dimension of 12.9 mm.

B, Follow-up scan shows the mass in a transverse 12-o’clock position with a decrease in tumor height to 1.4 mm 4½ years after iodine 125 plaque brachytherapy.

This lesion corresponds to the site of the choroidal melanoma and plaque placement.A radioactive episcleral iodine 125 (125I) plaque was surgically placed over the lesion and removed 1 week later.

The tumor height decreased to 2.3 mm by 1 year and remained at less than 2 mm in years 2 through 4½ on B-scan ultrasonography (Figure, B).At her last follow-up, the patient had a 4.5 × 3.0-mm bluish black flat lesion on the sclera (Figure, C).

Results of a dilated fundus examination showed a grayish black, minimally elevated choroidal lesion with surrounding retinal pigment epithelial atrophy but without subretinal fluid, hemorrhage, or hard exudates.

C, An external slitlamp photograph of the left eye shows a bluish black pigmented lesion on the sclera measures 4.5 × 3.0 mm (anterior-posterior by horizontal orientation).

The tumor decreased in height, and visual acuity remained stable at 20/30 OS.

Her choroidal melanoma was located at the 11- to 1-o’clock position, anterior to the equator but posterior to the ciliary body.


He was diagnosed as having nephropathy 5 years ago.

He presented with a history of bilateral, painless, progressive visual diminution for 2 years.

On examination, his best-corrected visual acuity was 20/200 in both eyes.

He had bilateral posterior subcapsular cataracts.

Intraocular pressures were 14 and 16 mm Hg in the right and left eyes, respectively, by Goldmann applanation tonometry.

Dilated ophthalmoscopy revealed a hypopigmented zone in the parafoveal macula (Figure).

Color vision testing revealed red-green deficiencies in both eyes.Advanced chloroquine retinopathy.

Fundus photographs of the right (A) and left (B) eyes showing hypopigmented zones concentric to the fovea consistent with chloroquine retinopathy.


The patient was otherwise healthy with normal development and growth.

She had no other medical problems and was not taking any medications.

The patient’s history was otherwise unremarkable; she had no pets, had no recent travel outside the United States, and her vaccinations were up to date.On physical examination, the patient had well-defined, follicular, pink, scaly papules coalescing into thin plaques on her knees (Figure 1A), elbows, and dorsal hands and feet, as well as diffuse, marked orange-red hyperkeratosis and hyperlinearity of her palms with clear demarcation at the wrists (Figure 1B).A, Well-defined follicular, pink, scaly papules coalescing into plaques on the patient’s knees.

The rash started as small pink bumps on her knees and elbows, spreading to her hands and feet and progressively thickening.

B, Marked orange-red hyperkeratosis of the patient’s palms.Initial treatment with hydrocortisone valerate cream, 0.2%, provided minimal improvement.


There was no family or personal history of bleeding disorders.

The epistaxis occurred 3 to 4 times a day and resolved with pressure.

He did not have facial pain, facial paresthesia, or visual changes.

Nasal endoscopy revealed a large, well-vascularized, polypoid mass filling the right anterior nasal cavity.

A computed tomographic scan showed a right nasal cavity mass (4.5 × 1.7 cm) extending to the posterior choana with opacification and bony remodeling of the right maxillary sinus.

The mass had heterogeneous intermediate signal intensity on T2-weighted magnetic resonance imaging (Figure, A).

Hematoxylin-eosin stain showed a sheet-like proliferation of epithelioid and polygonal cells with pale eosinophilic granular cytoplasm and relatively uniform vesicular nuclei (Figure, B).

Branching, staghorn-like blood vessels were scattered throughout the tumor.

The tumor cells stained positive for smooth muscle actin (Figure, C), muscle specific actin, Bcl-2, INI-1, and transcription factor E3 (Figure, D).

A, T2-weighted magnetic resonance imaging (MRI) scan of a right nasal cavity mass extending to the posterior choana with heterogeneous intermediate signal intensity.

B, A sheetlike proliferation of epithelioid and polygonal cells with pale eosinophilic granular cytoplasm and relatively uniform vesicular nuclei (hematoxylin-eosin, original magnification ×40).

C, The tumor cells stained positive for smooth muscle actin (original magnification ×20).

The patient was taken to the operating room for biopsy and possible excision of the nasal mass.

However, the lesion was found to have a narrow pedicle of attachment and was resected in its entirety.

As the lesion was biopsied, there was brisk bleeding.

The mass was based on the superior aspect of the nasal septum and cribriform plate.


His parents reported that he complained of left orbital pain just before they noticed the swelling, and he was treated with cold compresses with only slight improvement.

He had never experienced these symptoms before.

The patient had not experienced diplopia, change in visual acuity, nasal obstruction, change in oral intake, weight loss, recent upper respiratory infection, sick contacts, fevers, or chills.

At the time of presentation, the patient was breathing comfortably and was afebrile.

Findings from his head and neck examination were significant for a firm mass over the left zygoma and lateral infraorbital rim, 2 × 3 cm in diameter.

The mass was nontender, nonerythematous, and fixed to the underlying zygoma and lateral infraorbital rim.

Ophthalmology examination revealed intact extraocular movements, bilateral visual acuity of 20/50, and no evidence of afferent papillary defect.

Computed tomographic (CT) images showed a round, soft-tissue mass with osseous destruction and erosion into the zygoma, infratemporal fossa, inferolateral orbit, and maxillary sinus (Figure, A and B).

Concern for orbital involvement prompted magnetic resonance imaging (MRI) of the maxillofacial region (Figure, C and D).

He was taken to the operating room the following day for open biopsy.

A and B, Computed tomographic images of the maxillofacial region showing a round, soft-tissue mass.

C and D, Magnetic resonance imaging (MRI) scans of the maxillofacial region; C, T2-weighted MRI scan, axial view.

D, Postcontrast T1-weighted MRI scan, coronal view.


However, he used to wear a cap, which created pressure and friction on the upper part of both ears.

The nodules were painful on touch, especially while the patient was sleeping in a lateral position.

On examination, the free border of the helix of both the ears showed a few dull red, dome-shaped, firm nodules 2 to 3 mm in size with central crusting (Figure, A).

No cervical lymphadenopathy was observed.

One of the nodules on the right ear was excised; histopathologic features of the nodule are shown in the Figure, B.

It showed a sharply defined, centrally depressed ulcer covered by a hyperkeratotic parakeratotic scale, occasional bacterial colonies, and plasma.

The adjacent epidermis showed hyperplasia.

The base of ulcer showed eosinophilic degeneration of collagen and solar elastosis.

In addition, there were a few proliferating blood vessels and mild lymphomononuclear infiltrate.

Underlying cartilage was not seen in the section.

On serial sectioning, cartilage was seen and found to be normal.A, An ear with dull red, dome-shaped, firm nodules 2 to 3 mm in size with central crusting (arrowheads).

B, Histopathologic image (hematoxylin-eosin, original magnification ×100).


She first noticed the mass following an uncomplicated dental procedure.

It grew slowly for a few years but then stabilized.

She stated that it was sensitive to cold liquids but was otherwise asymptomatic.

Results from routine laboratory tests and hematologic markers were normal.

Examination revealed a roughly 4 × 2.5-cm, nontender, firm mass anterior to the right mandibular parasymphysis.

The mass was fully mobile relative to the underlying mandible and could be seen bulging into the gingivolabial sulcus.

There were no overlying skin or mucosal changes, and the skin and mucosa moved freely over the mass.

Computed tomographic (CT) imaging demonstrated a well-defined, hyperdense, mildly heterogeneous mass with few focal areas of fat attenuation along the inferior aspect of the lesion (Figure, A-C).

The mass was separate from the right mandibular parasymphysis without evidence of periosteal reaction or erosion into the underlying bone.

The surrounding soft tissues appeared unremarkable.

The mass was not fixed to any adjacent structures.A-C, Computed tomographic images.

Axial views of soft tissue (A) and bony windows (B) demonstrating an ossified hyperdense mass within the right gingivobuccal space with well-defined margins.

The patient was taken to the operating theater, where a firm, multilobulated, yellow mass was excised through a gingivolabial incision (Figure, D).

D, The intraoperative photograph shows the well-defined mass, just prior to removal, arising in the right gingivobuccal space, separate from the underlying mandible.


She was diagnosed with depression and began treatment with antidepressant medication.After 8 months, she had worsening complaints.

At that time, a family member reported that her memory problems had progressed.

She was no longer able to find misplaced objects, remember appointments, or handle financial affairs.

She was often subdued, her mood depressed, and her sleeping time increased.

Clinical examination at that time revealed a flattened affect and a 22 of 30 Mini-Mental State Examination score.

Findings were otherwise normal, and there were no focal neurologic deficits.

Basic laboratory workup results were unremarkable.

She was referred to a neurologist, who ordered contrast-enhanced magnetic resonance imaging (MRI) of the brain.The MRI (Figure) revealed extensive ill-defined areas of FLAIR (fluid-attenuated inversion recovery) and T2 signal abnormalities with additional enhancing nodular foci located within deep white matter structures, the basal ganglia, the caudate, the pons, and the midbrain.

Also identified was enhancement of the interpeduncular cistern and cerebral peduncles, suggestive of leptomeningeal involvement.Magnetic resonance images (MRIs) of the head obtained 8 months after patient’s first clinical visit.

A, Axial T2-weighted MRI of the head at the level of the lateral ventricles demonstrates extensive ill-defined areas of increasing signal with only minimal associated mass effect involving the frontal lobe white matter, greater on the left than on the right.


Episodes progressed in frequency and duration, occurring several times daily and lasting for hours.

His symptoms were prominent after activity and reliably triggered by straining with urination and terminated by lying supine.

The patient also reported a 1-week history of urinary retention requiring intermittent self-catheterization.

Neurological examinations both during and between episodes revealed symmetric patellar and Achilles hyperreflexia with bilateral crossed adductor response, but otherwise the results were normal.

A thoracic spine magnetic resonance imaging scan with fast imaging employing steady-state acquisition (FIESTA) sequences was obtained (Figure).

A dynamic computed tomographic myelogram demonstrated a lesion compressing the dorsal spinal cord at the T7 level (Video).Magnetic resonance imaging (MRI) of the thoracic spine using fast imaging employing steady-state acquisition (FIESTA) sequences.

The MRI scan with FIESTA sequences revealed a blurry spinal cord signal alteration at the T7-T8 level with deformation of the cord posteriorly (arrowhead).


Her medical history included hypertension and gastroesophageal reflux.

Her only operations in the past were nononcologic, nongastrointestinal procedures.On examination, her heart and lungs were normal.

She reported acute onset of pain that woke her up from sleep, was crampy in nature, and was localized to the right upper quadrant.

She denied any nausea or vomiting and there was no association with eating.

She reported similar pain over the previous month but not to the current severity.

There was no history of fevers/chills, diarrhea, melena, or hematochezia.

Her abdomen was soft but tender in the right upper quadrant.

Laboratory evaluation revealed a normal compete blood cell count, including a white blood cell count of 8900/µL (to convert to ×109 per liter, multiply by 0.001).

Results from a complete metabolic panel, including lipase, and from carcinoembryonic antigen and carbohydrate antigen 19-9 testing were normal.

Ultrasonography revealed asymmetric thickening of the gallbladder wall without pericholecystic fluid.

Computed tomographic scans of the abdomen with oral and intravenous contrast were obtained (Figure 1).Computed tomographic scan with coronal (A) and sagittal (B) views showing a soft-tissue mass associated with the fundus of the gallbladder.


She had no personal or family history of venous thrombosis and did not have any recent surgery, trauma, or admission to hospital.

Her long-term medications included fluoxetine, vitamin D, and hydrochlorothiazide.On examination, temperature was 38.3°C (101°F), blood pressure was 157/78 mm Hg, pulse rate was 82 beats per minute, and respiratory rate was 20 breaths per minute.

She had no leg swelling and no pain on calf palpation.

She had a regular heart rate with a mild systolic murmur; her jugular venous pressure was normal; and  lung auscultation revealed reduced air entry at the left base.

Laboratory testing results are reported in Table 1.

The clinical probability of PE was unlikely (Wells score).D-dimer test result is positive.

The attending physician raised the diagnosis of a pulmonary embolism (PE) among the differentials.

Imaging test is required.D-dimer test result is positive.

The diagnosis of PE is confirmed.D-dimer test result is positive but below her age-adjusted cutoff.

PE is ruled out.D-dimer test result is positive due to active malignancy.


The pain began after the patient began to play competitive basketball and was localized to her mid shin.

Dietary and menstrual histories revealed no abnormalities.

The single-leg hop test elicited severe localized pain.

Examination demonstrated focal tenderness along the distal mid shaft of the tibia anteriorly.

Radiographs were obtained for further evaluation (Figure).Radiography of the right lower leg.


He was diagnosed with bronchiectasis of unknown etiology 6 years ago and notes a worsening of his cough and dyspnea over the past few months.

His medications include albuterol inhaler, fluticasone-salmeterol inhaler, guaifenesin, and loratadine.Left, Axial computed tomography scan of the chest revealing mild bronchiectasis and ground glass inflammation.

He has a purpuric plaque on the right medial ankle and dorsal foot, with central vesicles (Figure, right).

The rash is tender to palpation, and there is associated nonpitting edema of the right foot and ankle.

A recent computed tomography scan of the chest revealed mild bronchiectasis and ground glass inflammation (Figure, left), and recent pulmonary function tests showed combined obstructive and restrictive defects.

Right, Purpuric plaque on the right medial ankle and dorsal foot, with central vesicles.On examination, the patient’s vital signs are normal.

Laboratory values are notable for a leukocyte count of 15 700 cells/µL, with 38% eosinophils (absolute count, 5950 cells/µL; normal, <350).

His platelet count was 272 000, and his hemoglobin level was 14.7 g/dL.

He has had no fevers, joint pain, recent trauma, or new exposures to the area.


The patient returned 1 week later with worsening cutaneous eruptions and new complaints of fever, weakness, and malaise.

Findings from physical examination were notable for discrete skin-colored to pink papules coalescing into plaques on her face, trunk (Figure, A and B), and all 4 extremities.

There was no scale or other epidermal change.

A punch biopsy for hematoxylin-eosin staining was performed (Figure, C).

Repeat physical examination revealed splenomegaly and axillary lymphadenopathy.

A peripheral blood smear (Figure, D) and other diagnostic tests were performed.A, Discrete skin-colored to pink papules on the abdomen.

B, Discrete papules coalescing into plaques on superior gluteal cleft.

C, Punch biopsy specimen (hematoxylin-eosin).

D, Peripheral blood smear (hematoxylin-eosin, original magnification ×100).


On physical examination, the patient was in severe discomfort with limited movement owing to cutaneous pain.

He was cachectic and had widespread erosions and tense and flaccid bullae with overlying crust affecting his trunk and extremities (including the palms and soles) but sparing his scalp (Figure, A).

He had severe stomatitis with localized hemorrhagic lesions, shaggy erosions on the buccal and gingival mucosa, and erythematous macules on his hard and soft palates (Figure, B).

Findings from laboratory tests, including a complete blood cell count and a comprehensive metabolic panel, were unremarkable except for mild anemia.

Results from a computed tomographic scan of the chest and neck showed adenopathy suspicious for lymphoproliferative disorder or potentially metastatic disease.

A punch biopsy for histopathologic examination (Figure, C) and direct immunofluorescence (DIF) was performed, and serum was tested for indirect immunofluorescence (IIF) on rodent epithelium (Figure, D).A, Erosions, tense and flaccid bullae, and overlying crust over the dorsal side of the foot.

B, Severe stomatitis and hemorrhagic crust.

C, Histopathologic image of a biopsy specimen showing suprabasal acantholysis and a dermal infiltrate of lymphocytes, histiocytes, and scattered eosinophils (hematoxylin-eosin, original magnification ×20).

D, Positive indirect immunofluorescence test of rat urinary bladder epithelium.


SLE had been diagnosed ten years previously after she presented with a positive antinuclear antibody titer (most recent titer, 1:1280), arthralgias, leukopenia, oral ulcers, and a malar erythematous eruption.

There was no history of discoid or tumid lupus lesions.

Physical examination revealed poorly demarcated erythematous plaques and nodules, tender to palpation, ranging from the distal thigh to the lower leg, adjacent to arthroscopy sites.

Lesions around the suture sites appeared more violaceous.

A punch biopsy of the lesion was performed (Figure, B and C).A, Clinical photograph of right knee demonstrating ill-defined erythematous plaques and nodules adjacent to 2 small incisional scars from previous arthroscopy.

B and C, Photomicrographs of punch biopsy specimen.

B, Hematoxylin-eosin, original magnification ×4.

C, Hematoxylin-eosin, original magnification ×10.

Initially, the lesions were thought secondary to soft-tissue infection and were treated with courses of clindamycin, vancomycin, and doxycycline.


His ocular history included enucleation of his right eye secondary to a traumatic ruptured globe 6 years previously.

He denied any systemic medical problems and was not taking any medications.

He reported that he first noticed a small lesion on the nasal conjunctiva of his left eye 6 months earlier; there was rapid growth during the last 2 months.

The mass became painful 4 weeks earlier and started to affect his vision 2 weeks earlier.

On examination, his visual acuity was no light perception OD (prosthesis) and light perception OS.

External examination revealed a well-fitting prosthesis in the right eye with a healthy upper and lower eyelid fornix.

An incisional biopsy was performed.Clinical photograph at presentation demonstrating a large, hemorrhagic, necrotic mass obscuring the entire globe and eyelids of the left eye.


Her medical history was significant for stage II breast carcinoma with positive lymph nodes, which was diagnosed and treated 5 years prior.

Visual acuity was 20/60 OD and 20/20 OS.

Ophthalmoscopy of the right eye revealed a 6 × 6-mm circumscribed serous macular detachment with turbid subretinal fluid.

Similar lesions were present in the nasal and inferior midperiphery (Figure, A and B).

Fluorescein angiography (FA) showed hyperfluorescent pinpoint hot spots with interspersed hypofluorescent patches in the areas of the lesions (Figure, C-F).

Notably absent on FA were retinal pigment epithelium (RPE) leakage, a smokestack pattern of subretinal leakage, or RPE gutter defects.

B-scan ultrasonography demonstrated choroidal thickening nasal to the optic disc (Figure, G); vascularity could not be assessed.

Optical coherence tomography (OCT) showed a localized serous macular detachment with a focal area of choriocapillaris infiltration and thickening near the nasal edge of the detachment (Figure, H).Fundus photograph (A-B) of the right eye demonstrates a serous macular detachment with yellowish choroidal discoloration.

A, Similar yellowish choroidal lesions (arrowheads) were present in the nasal and inferior midperiphery.

B, The enlarged image demonstrates the extent of serous macular detachment (arrowheads).

C, Wide-angle fluorescein angiography in the late venous phase of the right eye shows an area between the disc and fovea with scattered hyperfluorescent hotspots.

Magnified views of the macular lesion (D), nasal lesion (E), and inferior lesion (F).

G, B-scan ultrasonography of the right eye shows a subtle nonspecific choroidal thickening with medium internal reflectivity.

The arrowhead points to the choroidal infiltration by the metastatic tumor.


The child was otherwise well and no previous skin or nail conditions were known.

Her mother reported a sudden onset of brittle nails with cracks followed by painless sloughing of nails.

Initially only the fingernails had been affected, but the toenails soon showed the same pathology.

On clinical examination, most fingernails showed semilunar whitish grooves in the middle of the nail plate (Figure, A).

The toenails had transverse partial cracks of the proximal nail plate with distal onycholysis but normal proximal nail growth (Figure, B).Findings on clinical examination.

A, Fingernails showed semilunar whitish grooves in the middle of the nail plate.

B, Toenails had transverse partial cracks of the proximal nail plate with distal onycholysis.

The family was concerned and suspected an internal disease or vitamin deficiency.


He had no history of sinus disease or surgery and had no epistaxis, weight loss, or visual symptoms.

Computed tomography showed a 3.1-cm mass in the left ethmoid sinus and nasal cavity (Figure, A).

Positron emission tomographic scanning showed no evidence of metastasis.

Nasal endoscopy revealed a friable, polypoid mass in the ethmoid sinus with extension into the nasal cavity and superiorly to the skull base.

A biopsy specimen showed a heterogeneous tumor with small, angulated, blue cells with hyperchromatic nuclei, apoptosis, and mitosis, malignant glands lined by cuboidal cells with round nuclei, prominent nucleoli, and eosinophilic cytoplasm.

The glands had luminal mucin production (Figure, B).

Focally, there were also scattered round cells with abundant eosinophilic, refractile cytoplasm, features of rhabdomyoblasts (Figure, C).

These latter cells were strongly positive for desmin (Figure, D) and myogenin.

B, Blastema as indicated by the asterisk and malignant glands as indicated by the arrowhead.

C, Rhabdomyoblasts as indicated by the arrowheads (hematoxylin-eosin, original magnification ×40).

D, Desmin immunohistochemistry with strong positive staining in the rhabdomyoblasts (original magnification ×20).


Endoscopic nasopharyngoscopy revealed a large polypoid nasal mass occupying the left nasal roof and completely obstructing the nasal passage.

Contrast-enhanced T1-weighted magnetic resonance imaging (MRI) revealed a 1.7 × 3.5 × 2.6-cm soft-tissue mass within the left ethmoid cavity, eroding through the cribriform plate with adjacent dural enhancement (Figure, A).

Intraoperative pathologic examination showed polypoid spindle cell proliferation.

Histopathologic findings showed respiratory epithelium with submucosal vascular proliferation with no atypia among the lining endothelial cells.

The Figure, B, shows a central vessel surrounded by lobules of endothelial lined capillaries.

The Figure, C, highlights the endothelial cells of the numerous capillaries and the central vessel.A, Contrast-enhanced T1-weighted magnetic resonance imaging of a soft-tissue mass within the left ethmoid cavity.

B, Central vessel surrounded by lobules of endothelial-lined capillaries (hematoxylin-eosin, original magnification ×20).

C, Endothelial cells of the numerous capillaries and the central vessel on CD34 staining (original magnification ×20).


Although she had been experiencing a gradually increasing lumpy feeling on swallowing for at least 6 months, she did not experience of dysphagia, dyspnea, or pain.

Physical examination revealed a mass measuring approximately 4 × 3 cm and covered with normal mucosa on the posterior oropharyngeal wall.

This tumor was firm, nontender, and firmly adherent (Figure, A).

Computed tomography (CT) showed a segmented bony lesion located at the odontoid process and the body of the C2 vertebra (Figure, B).

This mass showed high signal intensity with a hypointense rim in both T1- and T2-weighted magnetic resonance images (MRI) (Figure, C and D, respectively).

Polysomnography was performed and showed an apnea-hypopnea index of 58.7/h and a minimal oxygen saturation of 76%, which led to a diagnosis of obstructive sleep apnea.

Hence, we performed total excision of the mass using a per-oral approach.A, Photograph of an oropharyngeal mass.

The mass was considered causative of obstructive sleep apnea.


She was otherwise healthy, with no medical or surgical history.

She had no associated pain, difficulty swallowing, or difficulty breathing.

Findings from her physical examination were unremarkable with the exception of a 2 × 2-cm firm, nontender, submucosal posterior soft palate mass just left of the uvula.

There was no overlying ulceration or discoloration.

Prior to presentation, a computed tomographic scan and biopsy had been performed at an outside hospital.

Magnetic resonance imaging (MRI) of the face with contrast was also performed (Figure, A-C).

Imaging showed a well-circumscribed solid lesion with no evidence of infiltration of soft-tissue structures, the pterygopalatine fossa, or palatine nerve foramina.

The lesion was resected via a transoral approach and was easily dissected from the surrounding tissue (Figure, D).


The patient had a history of prostate cancer treated with prostatectomy 4 years previously without evidence of recurrence and melanoma of the posterior neck treated with wide local excision, in addition to non–insulin-dependent diabetes mellitus and hyperlipidemia controlled with oral medications.

He does not smoke and has been in a monogamous marriage for more than 30 years.

The rash initially started 4 years prior as a dime-sized, pink, scaly area in the right inguinal crease and was pruritic.

Since then, the rash had failed to resolve and the affected area had increased in size to its present dimensions.

Examination revealed a multifocal scaly, pink-red beefy plaque in the right inguinal crease extending from the superior aspect of the scrotum to the gluteal crease measuring approximately 6 × 7 cm in total area with some areas of excoriation and superficial open wounds.

An additional area of macular erythema extended superiorly along the inguinal crease toward the medial thigh.

No lymphadenopathy of the inguinal region was noted.

Results of a biopsy at the time were consistent with lichen simplex chronicus, and treatment with topical steroids was initiated.


An abdominal magnetic resonance image is shown in Figure 1A.

Laparotomy exposed a giant multinodular mass (Figure 1B).A, Abdominal magnetic resonance image.

Given the symptoms and magnetic resonance imaging findings, the patient was eventually taken to the operating room.

B,Intraoperative photograph of the giant multinodular mass occupying most of the upper abdomen.


She had previously lived in Beijing, China.

During that evaluation, the patient reported that she had run in areas with dog feces on the ground and had eaten sheep meat (notably, not organ meat); she denied consuming canine meat.

In 2011, she was evaluated in a Chinese clinic for abdominal bloating and moderate left upper quadrant pain; she was offered a splenectomy, but she declined and pursued a second opinion at a hospital in the United States in 2012.

Results of serologic tests for Echinococcus antibody IgG were negative.

Results of a CT scan were remarkable for a 9.7 × 9.0 × 10.5-cm anterior cyst and a smaller posterior cyst measuring 4.6 × 4.7 × 3.8 cm, both with rim calcifications (Figure 1).A large splenic cyst with calcified capsule is present in the anterior aspect of the spleen, with a smaller cyst in the posterior aspect of the spleen, also with calcified capsule.

Arrowheads indicate the location of the cysts.

Under higher resolution, debris is noted within the larger cyst.


A glioma of the optic chiasm was treated 20 years prior with chemotherapy and radiation.

Medications included analgesics, cyclobenzaprine, sumatriptan, ondansetron, divalproex sodium, gabapentin, furosemide, somatotropin, potassium chloride, vitamin D, and estrogen.

Sequelae included chronic headaches, anterior hypopituitarism, and hydrocephalus necessitating a ventriculoperitoneal shunt.

Mucosae were moist and jugular venous pressure was not well seen.

Her cardiopulmonary and abdominal examinations were normal, peripheral edema was absent, and sensorium was clear.


He reported being diagnosed with dermatitis herpetiformis more than 20 years ago and receiving sulfapyridine and dapsone for treatment.

He stopped taking these medications because of limited benefit and concern about nephrotoxicity.

Physical examination revealed diffuse erosions with grouped papules and vesicles on his bilateral extremities, elbow and knee extensors, chest, back, buttocks, face, and scalp (Figure 1).

Recently, extensive skin disease and pruritus developed.

He noted 20 years of chronic diarrhea, which improved with a gluten-free diet, in addition to chronic renal insufficiency, hypothyroidism, and aortic stenosis.

Examination also revealed extensive excoriations, as well as superficial honey-colored crusts.

Since then, he was treated with periodic courses of oral antibiotics and oral corticosteroids.


She reported no chest pain, dyspnea on exertion, presyncope, orthopnea, paroxysmal nocturnal dyspnea, or pedal edema.Physical examination demonstrated blood pressure of 140/76 mm Hg and a heart rate of 72/min.

Carotid upstrokes were slightly delayed with a transmitted murmur.

Cardiac examination demonstrated a regular rate and rhythm, normal S1, 3/6 harsh, late-peaking systolic murmur that radiated to the carotids with soft A2, 2/4 diastolic decrescendo murmur at the left lower sternal border, and 2/6 holosystolic murmur at the apex.

There was no edema.A 2-D and Doppler transthoracic echocardiogram (TTE) was performed to assess valvular function (Table; Interactive).The patient has functional mitral regurgitation secondary to a dilated cardiomyopathy.The patient has moderate aortic valve stenosis with the severity overestimated by Doppler measurements.The patient has severe aortic valve stenosis with preserved left ventricular systolic function.


Past medical history was significant only for well-controlled hypertension treated with irbesartan (300 mg daily) and poor vision in the left eye for many years attributable to presumed inactive ocular histoplasmosis.On presentation, the patient appeared well, with blood pressure of 140/70 mm Hg and a regular heart rate of 75/min.

He also noticed rhythmic pulsing sounds in both ears during the past month.

Best corrected visual acuity was 20/200 (right eye) and counting fingers at 1 foot (left eye).

Direct ophthalmoscopy revealed severe bilateral optic disc edema, peripapillary hemorrhages, and absent spontaneous venous pulsations (Figure 1).

Findings from a full neurologic examination were otherwise normal.


At the time of consultation, the patient was taking oral treatment with duloxetine hydrochloride, clonazepam, oxcarbazepine, fentanyl, sulpiride, zopiclone, omeprazole magnesium, and baclofen.

He also admitted to self-administering subcutaneous injections of meperidine, 100 mg 4 times per day, for the past 3 years, at different sites, including the deltoid areas and abdomen.

Physical examination disclosed a large and deep, irregularly shaped, cutaneous ulcer on the dorsum of his right forearm.

Necrotic tissue and muscle exposure was seen at the base of the ulcer (Figure, A).

Woody induration of skin on both forearms and on the abdominal region was also observed.

Bilateral contracture of deltoid, triceps, and biceps muscles was noted.

Active and passive range of motion was restricted at the shoulders and elbows.

No signs of joint inflammation were seen.

Growth from culture specimens taken from the ulcer was negative for bacteria, mycobacteria, and fungal organisms.

His serum creatinine kinase level was raised (192 U/L; reference range, 0-174 U/L), but test results for complete blood cell count; erythrocyte sedimentation rate; antinuclear antibody, rheumatoid factor, aspartate aminotransferase, alanine aminotransaminase, and aldolase levels; and serum electrophoresis were all within normal limits.

A wedge biopsy from the indurated skin of the abdominal region was performed (Figure, B and C).

(To convert creatinine kinase to microkatals per liter, multiply by 0.0167.

)A, Clinical photograph of the large, deep, irregularly shaped, cutaneous ulcer on the dorsum of the right forearm.

Three small ulcers are seen adjacent to the larger ulcer.

B and C, Histologic images of a wedge biopsy specimen from the indurated skin of the abdominal region (hematoxylin-eosin).


He recalled that a small lesion had appeared approximately 8 months prior, and over the past several months he had noted significant growth and pain.

A nonpruritic rash had also become apparent on his forearms and thighs.

Physical examination showed a 2-cm macerated pedunculated growth on the left buttock within the intergluteal cleft (Figure, A).

An erythematous rash was noted on the forearms and thighs with fine scale present at the wrists.A, Photograph of a 2-cm macerated pedunculated growth on the left buttock.

C, Histopathologic image of immunohistochemical staining for spirochetes (original magnification ×400).A shave biopsy specimen of the exophytic growth on the buttock revealed marked epidermal hyperplasia (Figure, B) with a dense dermal infiltrate containing lymphocytes and plasma cells.

B, Histopathologic image of an exophytic growth on the buttock with marked epidermal hyperplasia (hematoxylin-eosin, original magnification ×40).