Affiliation:
1. Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada
2. Division of Dermatology, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada
Abstract
Segmental Darier’s disease is an uncommon subtype of Darier’s genodermatosis, resulting from a mutation in the ATPase type 2 during early embryogenesis. It typically presents as a persistent, pruritic papular eruption following the lines of Blaschko. Histopathology of Darier’s disease demonstrates acantholysis, dyskeratosis, and corps ronds. First-line treatment includes topical retinoids, calcineurin inhibitors, and synthetic vitamin D analogues. Severe disease may require systemic therapy with oral retinoids, immunomodulators, magnesium, and low-dose naltrexone. Segmental Darier’s disease is important to recognize both clinically and histologically as it may resemble other acantholytic Blaschkolinear dermatoses and should be considered in individuals presenting with a chronic localized papular eruption in a Blaschkoid distribution. Herein, we present a case of a 48-year-old male with segmental Darier’s disease who improved significantly following acitretin treatment.