A Rare Presentation Of Type 1 Segmental Variant Of Darier’s Disease In Blaschkoid Pattern - A Case Report

Abstract

Darier disease (DD), is an uncommon, slowly progressive, autosomal-dominant skin disorder characterized by multiple keratotic papules, loss of adhesion between epidermal cells, and abnormal keratinization. Here we report a 38 year old male who presented with intense itchy hyperpigmented, hyperkeratotic papules and plaques in blaschkoid distribution over right side of face , post auricular region,  shoulder, trunk and posterior aspect of right lower limb in a chronic and relapsing course since adolescence for the past 20 years. Based on clinical and histopathological evaluation, diagnosis of type 1 segmental Darier’s disease was made. The segmental variant accounts for only 10% of the total cases of Darier’s disease, an uncommon disease, with only a very few cases being reported in the literature.