S003 CPC Self-Assessment

Transcription

1 S003 CPC Self-Assessment Alina G. Bridges, D.O. Associate Professor Program Director, Dermatopathology Fellowship Department of Dermatology, Division of Dermatopathology and Cutaneous Immunopathology Mayo Clinic, Rochester, MN

2 Disclosures Relevant Financial Relationships None Off Label Usage Yes

3 CASE 1 I itch and nothing helps!!

4 CASE 1 54 y/o F with severe pruritic eruption x 1.5 years Admitted to Dermatology inpatient service for wet dressings Outside skin biopsies non-specific dermatitis DIF? DH (negative anti-gliadin and TTG Abs, duodenum biopsy negative for celiac dz) Prior treatments: - Topical corticosteroids (poor response) - Dapsone gel (poor response) - UVB (some benefit) - Dapsone 50 mg QD x 8 months (initially helpful) - Systemic corticosteroids ( 30 day course w/ taper) - Hydroxyzine and doxepin (some benefit)

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6 2011 MFMER slide-6

7 2011 MFMER slide-7

8 What is your diagnosis? A. Scabies B. Prurigo Nodularis C. Dermatitis Herpetiformis D. Bullous Pemphigoid E. Lymphoma

9 Pruritus Evaluation Normal or negative: CBC, peripheral smear, ESR, SPEP, IgA, IgM, IgG, TSH, ANA, gliadin Ab, endomysial Ab, TTG, Hep B/C, HIV, CXR AST 51 and ALT 111: known steatohepatitis Skin swab: Methicillin sensitive Staph aureus Biopsies for H&E and DIF obtained

10 DIF: Linear deposition of IgG along BMZ IIF: 1:640 BMZ titer on monkey esophagus with epidermal pattern on salt-split skin Elevated BP180 (90) and BP230 (90) Final diagnosis: Bullous pemphigoid (pemphigoid nodularis)

11 Pearl: Atypical presentations of bullous pemphigoid 20% non-bullous Eczematous, dyshidrosiform, urticarial, erythrodermic, nodular, lichenoid, or targetoid Include evaluation for autoimmune bullous disorders in erythroderma and pruritus work-up

12 Pruritus Xerosis Dermatitis Hypersensitivity reaction Dermatophytosis Folliculitis Scabies Autoimmune bullous disease Liver disease Renal disease Thyroid disease Anemia Lymphoproliferative disorders

13 Laboratory evaluation of patients with bullous disease Histopathology Early intact vesicle Direct immunofluorescence Normal perilesional skin Blood/serum Indirect immunofluorescence (on monkey esophagus, rat bladder, SSS); TTGs/ endomysial antibodies; ELISA for target antigens (BP180/230; desmogleins 3 and 1)

14 CASE 2: Mystery diagnosis

15 52 year old male with generalized pruritic violaceous, keratotic papules in a linear and reticulate pattern on the trunk and extremities with oral ulcers X 4 years

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22 EVALUATION Evaluated extensively at various institutions including GWU, CC, JHU, and NIH Multiple skin and oral biopsies No new meds prior to eruption developing?plc, Bechet syndrome Extensive laboratory evaluation normal or negative CBC w/ diff; chemistry 20 panel; ESR, CRP; IgA, IgM, IgG, IgD, IgE; total C, C3, C4; ANA, ENA, ANCA; syphilis & hepatitis serologies; HIV; HHV6, EBV; CK, LDH, PTH, Ca, lipid profile, SPEP, ferritin; leukemia/lymphoma phenotype; vitamins B12 & D Negative DIF, IIF, BP180/230, desmogleins 3 & 1, celiac disease serology Treatment Topical & systemic corticosteroids, oral antihistamines, valacyclovir, adalimumab, ustekinumab, MTX, hydroxychloroquine, cyclosporine, topical tacrolimus, acitretin, NBUVB Currently on PUVA

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26 What is the most likely diagnosis? A. Lichen planus B. Lichenoid drug reaction C. Secondary syphilis D. Keratosis lichenoides chronica E. Pityriasis lichenoides

27 Keratosis Lichenoides Chronica (KLC) or Nekam Disease Rare disorder Unlike adult onset, pediatric disease onset may be familial/ar inheritance Characterized by violaceous papules in a linear and reticulate pattern on the trunk, flanks, buttocks and extremities as well as a seborrheic dermatitis-like facial eruption Oral ulceration, alopecia and nail involvement may occur Histopathology shows a lichenoid interface dermatitis with alternating areas of atrophy and acanthosis and focal parakeratosis with neutrophils CPC is required to make this diagnosis Course is chronic and progressive Refractory to many different treatment modalities Authentic and distinct entity, but many purported cases are LP, LE or LSC.

28 This disorder may be associated with which of the following conditions: A. Hyperthyroidism B. Lymphoproliferative disorders C. Hepatitis C infection D. Inflammatory bowel disease E. Connective tissue disease

29 Treatment Options Wet dressings with 2.5% hydrocortisone & 0.1% triamcinolone creams Phototherapy with oral retinoids Metronidazole 500 mg po bid X 2 months Lenalidomide 5-10mg daily Mycophenolate mofetil, azathioprine (TMPT levels normal), dapsone (G6PD level normal); Tofacitinib (Janus kinase inhibitor) Photodynamic therapy

30 Thank you! THANK YOU!

31 Comments/questions?