Abstract:

A 42-year-old man with history of Behcet’s disease treated with adalimumab presented to dermatology for evaluation of ‘abnormal skin’ on his right chest. On initial evaluation, the patient’s skin appeared unremarkable with no evidence of erythema, pigment alteration, or textural changes. Upon palpation, the tissue was doughy and pliable. Mechanical manipulation resulted in a rippled appearance of the skin that remained fixed until manually smoothed. A punch biopsy demonstrated scant perivascular lymphocytic infiltrate with rare eosinophils and increased dermal mucin. Movat’s pentachrome and Verhoeff–Van Gieson stains highlighted loss of elastic fibers in the papillary dermis and less prominent elastic fiber fragmentation in the reticular dermis. A biopsy of unaffected skin was taken for comparison. The control skin displayed retained intact elastic fibers in the papillary and reticular dermis with increased dermal mucin. Papillary dermal elastolysis is a rare entity occurring within a spectrum of diagnoses including fibroelastolytic papulosis and pseudoxanthoma elasticum-like papillary dermal elastolysis. This case represents a unique clinical presentation manifesting as rippled skin. Papillary dermal elastolysis may present as an isolated finding, associated with medications, or systemic disease. There are no reports of association with Behcet’s disease or adalimumab. Papillary dermal elastolysis most commonly presents with atrophic papules or plaques. Our case highlights a distinctive clinical variant of elastolysis to add to the literature.

Financial Disclosure:
No current or relevant financial relationships exist.

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Tanya Lindenmuth, DO, MS