Abstract

A 23-year-old woman with a history of interstitial lung disease, positive ANA, and weakly positive anti-Scl-70 presented with periumbilical blisters. Biopsy revealed a subepidermal split with eosinophils and neutrophils and suprabasilar acantholysis. Intercellular C3 on direct immunofluorescence (DIF) was suggestive of pemphigus vulgaris (PV). She subsequently developed a tense vesicle on her right shin. Repeat biopsy only demonstrated intraepidermal blister with re-epithelialization of blister floor.

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