Clinicopathologic Self- Assessment S006...Crusted (Norwegian) Scabies •All immunosuppressants stopped •Treatment: •Permethrin -> x2, 1 week apart •Ivermectin -> x2, 2 weeks

Clinicopathologic Self-Assessment

Melissa Piliang, MD

Cleveland Clinic

Dermatology and Pathology

Case 1

Biopsy showed:

• Scalp:• Perifollicular inflammation with

interface dermatitis

• Face:• Interface dermatitis with extensive

melanoderma

Diagnosis?

A. Acne

B. Seborrheic dermatitis

C. Lichen planopilaris

D. Lichen planus pigmentosa

E. C and D

Lichen Planopilaris and Lichen Planus Pigmentosa• Treatment:

• Topical steroids

• Oral prednisone

• Hydroxychloroquine

Persistent Rash and Extreme Pruritus

• Added:• Methotraxate

• Azathioprine

• Antihistamines

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

‘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….

Crusted (Norwegian) Scabies

• All immunosuppressants stopped

• Treatment:• Permethrin -> x2, 1 week apart

• Ivermectin -> x2, 2 weeks apart

Outcome

• LP Pigmentosa faded

• Itch dramatically improved

• Persistant• Mild scalp itch

• Scalp dermatitis

• Repeat scalp biopsy -> LPP without scabies

Clinical Infectious Diseases. 54(6):882;2012

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

Case 2

• 70 year old man

• Rheumatoid arthritis • Low dose prednisone

• Granular cell leukemia (in remission)

• Admitted for tender erythematous rash on arm

• Rapidly spread to all extremities

• Condition deteriorated• Fever

• Confusion

• Transferred to ICU

Blood Culture

• ‘Yeast’

What category of infection is most likely?

A. Bacteria

B. Fungus

C. Protozoa

D. Algae

E. Candida

What category of infection is most likely?

A. Bacteria

B. Fungus

C. Protozoa

D. Algae

E. Candida

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

PAS

The Lesson

• Always biopsy!

• Tissue cultures!

Case 3

• 30 year old man

• 6 month h/o oral ulcers

• Weight loss

• Felt unwell

• Unable to eat due to pain

History

• Prior outside biopsy showed acute and chronic granulomatous infiltrate

Next step?

A. Start prednisone

B. ANCA’s

C. Tissue culture

D. Repeat biopsy

E. CT chest

Reasonable next steps include all of the following except?A. Start prednisone

B. ANCA’s

C. Tissue culture

D. Repeat biopsy

E. CT chest

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

History

• Working dx: Granulomatosis with Polyangiitis (formerly Wegener’s granulomatosis)

• Treated with high dose prednisone (40-60 mg daily)

Work-up - Positives

• Leukopenia

• Anemia

• T-cell deficiency

• Hypoalbuminemia

• Endoscopy – superficial esophageal erosions

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

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

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

Histoplasmosis

Organisms surrounded by clear space Packed in histiocytes2 to 5 μm in diameter Thick cell wall GMS + and PAS +

Histoplasmosis

Further Work-up

• Bone marrow biopsy• Histoplasmosis

• Esophageal biopsy• Histoplasmosis

• Tissue culture• Histoplasmosis capsulatum

Treatment

• Amphotericin B

• Intraconazole

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)

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

Histoplasmosis

• Calcified lung nodules, similar to TB

• Fibrosing mediastinitis

• Ocular involvement: • Scarring of retina

• Subretinal hemorrhage

• Leads to blindness (like macular degeneration)

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

Thank You pilianm @ccf.org