A Compendium of Late-onset Darier with an Unusual Report

Abstract

Darier disease is an autosomal dominant inherited skin disorder with complete penetrance and variable expressivity due to mutation in the ATP 2A2 gene. It usually develops from childhood and persists through adolescence. Late onset of this genodermatosis has been infrequently reported and found to have minimal findings compared to the classical cases. Classical disease presents with greasy keratotic yellowish-brown papules over the seborrheic areas with palmar pits and nail changes. We hereby report a case of late onset comedonal darier disease with extensive hypopigmented lesions, involvement of palms and nails which is rarely reported in this setting. A thorough review of literature was done and a compendium prepared of late onset darier and no other case report was found to have such extensive involvement as the case reported. It will be interesting to compare more such late onset cases to see if they have a common pattern which can suggest them to be a separate entity from the classical darier.