Late onset Darier’s Disease with Palmoplantar Keratoderma: A Case Report

Abstract

Darier’s disease is an infrequently encountered autosomal dominant condition characterised by complete penetrance but variable expression. It is characterised by abnormal keratinisation and epidermal adhesion deficiency, clinically presenting as greasy keratotic or warty papules and plaques, primarily affecting seborrheic regions and flexures. Additional manifestations include palmar pits, nail abnormalities, cobble-stoning of the oral mucosa, and even neuro-psychiatric issues. In extremely rare cases, mucosal involvement can occur in the oral, laryngeal, Oesophageal, or rectal areas. Familial Darier’s disease typically emerges during puberty. Here, the authors present a unique case of 55 years old male patient of Darier’s disease with delayed onset, despite a significant family history of the condition. The clinico-pathological aspects, highlighting the presence of palmo-plantar keratoderma are also discussed. Treatment included the application of topical hydrocortisone 1% cream twice daily for two weeks, then once daily for two weeks along with dexpanthenol once daily for eight weeks.

Keywords

• hyperkeratotic papules
• photodermatitis
• pruritis