Diagnosis? 2/17/2018. Case year old female. F045 Dermpath Case Challenge

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1 Case 1 75 year old female F045 Dermpath Case Challenge Tammie Ferringer, MD Depts of Dermatology and Pathology Danville, PA tferringer@geisinger.edu 1 year history of undiagnosed joint pain and 6 month history of redness, swelling and pain of the dorsal feet and hand Favor erythromelalgia, may also consider pernio I have no relevant relationships with industry. Left ankle 1

2 Differential Inflammatory morphea Necrobiosis lipoidica IgG4 related sclerosing disease Plasma cell dyscrasia Acrodermatitis chronica atrophicans CD34 IgG4 Kappa and lambda CD34 interstitial loss Rare IgG4 positive plasma cells Kappa and lambda normal ratio Additional Clinical History Traveled to France in 1998 Acrodermatitis Chronica Atrophicans Follow-Up LYME IGG/IGM AB POSITIVE (A) NEG Final LYME IGG/IGM AB VALUE >12.4 (H) <0.91 INDEX Final LYME G/M COMMENT Final 18KDA IGG PRESENT (A) XDET Final 23KDA IGG NOT DETECTED XDET Final 28KDA IGG PRESENT (A) XDET Final 30KDA IGG PRESENT (A) XDET Final 39KDA IGG PRESENT (A) XDET Final 41KDA IGG PRESENT (A) XDET Final 45KDA IGG PRESENT (A) XDET Final 58KDA IGG PRESENT (A) XDET Final 66KDA IGG PRESENT (A) XDET Final 93KDA IGG PRESENT (A) XDET Final LYME WB INTERP. IGG LWBGN Final PRESUMPTIVELY POSITIVE: IGG ANTIBODIES PRESENT TO ANY 5 OR MORE OF THE FOLLOWING 10 SIGNIFICANT BANDS: 18,23,28,30,39,41,45,58,66 OR 93 kd 2

3 3 mos s/p 4 week course of doxycycline her cutaneous and rheumatologic symptoms resolved, discoloration improved and she can wear her wedding band Nat Rev Dis Primers. Author manuscript; available in PMC 2017 August 02. Acrodermatitis Chronica Atrophicans Red to violet distal extremities with swelling Can be sclerodermatous or lichen sclerosus-like Late atrophic lesions resemble crumpled tissue paper Mainly in Europe due to B. afzelii Older women 20% have history of EM 6 months to 8 years prior Untreated does not tend to resolve and fibrosis and atrophy can develop ACA vs Morphea ACA vs Morphea CD34 CD68 3

4 Epidermal atrophy Focal vacuolar Bandlike or diffuse dermal infiltrate Plasma cells of variable density Altered collagen Interstitial granulomatous Telangiectasia Take Home Swelling of the distal extremity with bluish red plaques and diffuse infiltrate of histiocytes and plasma cells, consider ACA Can mimic morphea, lichen sclerosus and mycosis fungoides May be coming to an office near you Case 2 30 year old male Diffuse erythematous papular and vesicular rash 4

5 Disseminated zoster? VZV Additional History 11/5 Presented to ER for coughing and shortness of breath along with drooping of right eyelid and double vision CT head: negative 11/6 Requested to return to ER for elevated D-dimer CT chest/abdomen: abdominal mass and liver lesions Lost 20 pounds in 5 weeks 11/10 Rash started 5 days ago on abdomen, back and groin so PCP started valtrex 5

6 Additional History 11/21 Worsening SOB and rash Admitted to outlying hospital Inconclusive liver biopsy Transferred to GMC based on telederm consult C3 6

7 IgG Additional History Lymph node and liver biopsy suspicious for malignancy possibly of histiocytic/dendritic cell origin BM negative Paraneoplastic Pemphigus Most with NHL but also CLL, Castleman disease, thymoma, Waldenstrom Usually presents with severe painful oral ulcers Can involve conjunctiva with symblepharon and lungs with obstructive disease Paraneoplastic Pemphigus Many different autoantibodies to proteins of the plakin family, envoplakin (210-kd), periplakin (190-kd), bullous pemphigoid antigen I (230-kd), desmoplakin I (250-kd), desmoplakin II (210-kd), plectin (500-kd), and alpha2- macroglobulin-like 1 (170-kd) Can also exhibit antibodies to antigens associated with pemphigus vulgaris (desmoglein 3, 130-kd) and pemphigus foliaceus (desmoglein 1, 160-kd) PNP can be herpetiform Take Home Oral involvement is not always first or the most severe Pemphigus can show a punctate intercellular pattern on DIF 7