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Clinicopathologic Self-Assessment
Melissa Piliang, MD
Cleveland Clinic
Dermatology and Pathology
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Case 1
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Biopsy showed:
• Scalp:• Perifollicular inflammation with
interface dermatitis
• Face:• Interface dermatitis with extensive
melanoderma
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Diagnosis?
A. Acne
B. Seborrheic dermatitis
C. Lichen planopilaris
D. Lichen planus pigmentosa
E. C and D
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Lichen Planopilaris and Lichen Planus Pigmentosa• Treatment:
• Topical steroids
• Oral prednisone
• Hydroxychloroquine
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Persistent Rash and Extreme Pruritus
• Added:• Methotraxate
• Azathioprine
• Antihistamines
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Still Miserably Itchy….
• What would you do next?A. Add azathioprine
B. Add doxepine
C. Do another biopsy
D. Admit to hospital
E. Tell him it is all in his head
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‘Itch Crisis’
• After many phone calls
• Went to ER (on his own)
• Admitted for ‘itch crisis’ with goal ‘to control itch’
• Another biopsy was performed….
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Crusted (Norwegian) Scabies
• All immunosuppressants stopped
• Treatment:• Permethrin -> x2, 1 week apart
• Ivermectin -> x2, 2 weeks apart
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Outcome
• LP Pigmentosa faded
• Itch dramatically improved
• Persistant• Mild scalp itch
• Scalp dermatitis
• Repeat scalp biopsy -> LPP without scabies
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Clinical Infectious Diseases. 54(6):882;2012
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Key Points
• Itch crisis? Think scabies
• Patients can have 2 things
• Scrape!
• Biopsy (and re-biopsy) diseases that fail to respond to treatment
• Scabies is a humbling disease to treat
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Case 2
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• 70 year old man
• Rheumatoid arthritis • Low dose prednisone
• Granular cell leukemia (in remission)
• Admitted for tender erythematous rash on arm
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• Rapidly spread to all extremities
• Condition deteriorated• Fever
• Confusion
• Transferred to ICU
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Blood Culture
• ‘Yeast’
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What category of infection is most likely?
A. Bacteria
B. Fungus
C. Protozoa
D. Algae
E. Candida
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What category of infection is most likely?
A. Bacteria
B. Fungus
C. Protozoa
D. Algae
E. Candida
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Protothecosis
• Localized or disseminated infection
• Algae
• Sporangia are thick walled spherical bodies often in cytoplasm of giant cells
• Many internal septations with endospore • Classic morula appearance
• Nonbudding
• Prominent wall
• Inflammation may be sparse
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PAS
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The Lesson
• Always biopsy!
• Tissue cultures!
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Case 3
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• 30 year old man
• 6 month h/o oral ulcers
• Weight loss
• Felt unwell
• Unable to eat due to pain
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History
• Prior outside biopsy showed acute and chronic granulomatous infiltrate
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Next step?
A. Start prednisone
B. ANCA’s
C. Tissue culture
D. Repeat biopsy
E. CT chest
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Reasonable next steps include all of the following except?A. Start prednisone
B. ANCA’s
C. Tissue culture
D. Repeat biopsy
E. CT chest
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Reasonable next steps include all of the following except?A. Start prednisone
B. ANCA’s
C. Tissue culture
D. Request special stains
E. CT chest
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History
• Working dx: Granulomatosis with Polyangiitis (formerly Wegener’s granulomatosis)
• Treated with high dose prednisone (40-60 mg daily)
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Work-up - Positives
• Leukopenia
• Anemia
• T-cell deficiency
• Hypoalbuminemia
• Endoscopy – superficial esophageal erosions
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Which test is more likely to lead to diagnosis?
A. HIV
B. CT scan head, neck, chest
C. Bone marrow biopsy
D. Skin biopsy with tissue culture
E. ANCA’s
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Which test is more likely to lead to diagnosis?
A. HIV
B. CT scan head, neck, chest
C. Bone marrow biopsy
D. Skin biopsy with tissue culture
E. ANCA’s
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Work-up - Normal
• HIV negative – multiple times
• Blood cultures – negative
• CXR – normal
• Imaging – showed ulcers, but no lesions outside oral/nasal cavity
• Renal function – normal
• ANCA’s negative
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Histoplasmosis
Organisms surrounded by clear space Packed in histiocytes2 to 5 μm in diameter Thick cell wall GMS + and PAS +
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Histoplasmosis
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Further Work-up
• Bone marrow biopsy• Histoplasmosis
• Esophageal biopsy• Histoplasmosis
• Tissue culture• Histoplasmosis capsulatum
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Treatment
• Amphotericin B
• Intraconazole
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Histoplasmosis
• Airborne pathogen
• Inhalation of spores
• Soil contaminated with bat or bird excrement
• Farmers, gardeners, construction workers, HVAC, cave explorers
• Ohio River Valley: OH, IN, MO, MS (+ skin tests in 80% of population)
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3 Forms
• Acute or primary: – Flu-like symptoms
– Most recover without treatment
– Many unaware of infection
• Chronic: – Pulmonary
– Can be fatal
• Disseminated: – Extra-pulmonary involvement
– Often fatal
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Histoplasmosis
• Calcified lung nodules, similar to TB
• Fibrosing mediastinitis
• Ocular involvement: • Scarring of retina
• Subretinal hemorrhage
• Leads to blindness (like macular degeneration)
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Key Tips
• Histoplasmosis often causes oral ulcers
• Beware neutrophilic (acute, suppurative) and granulomatous inflammation – Ask for special stains!
• The majority of patients unaware of exposure
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Thank You pilianm @ccf.org