TMSS Medical College Journal

Darier’s disease or keratosis follicularis is an uncommon disorder of keratization which is autosomal dominant and have variable penetrance. It begins in adolescence or early adult life with discrete or confluent, brown or skin colored foul smelling, greasy, crusted, dirty looking, papules or plaques at the seborrhoeic sites, such as scalp, face, nasolabial folds, chest, back, axillae & groins. Palms & soles also may show minute pits & dorsal of hands shows small flat discrete papules. Sometimes cobblestone papules occur on the palate. Nails may have angular nicks. Sometimes Darier’s disease is asymptomatic. It may run a chronic course with exacerbation in summer & relative remission in winter. Histopathology shows supra-basal acantholysis with dyskeratosis. Treatment includes avoidance of triggering factors like- sweating, frictions, UVB etc. and topical emollients such as- urea & lactic acid, topical Corticosteroid; topical & systemic retinoid can be given. Here we present a case of Darier’s disease with typical skin involvement. Key words :

Darier’s disease (DD), or keratosis follicularis is a rare genodermatosis was described independently by ‘White and Darier in 1889. It is an autosomal dominant disease with variable expressivity. It is more common in female than male and the total ratio is 1 in 50,000-1,00,000 people. Darier’s disease is clinically manifested by hyperkeratotic papules primarily affecting head, neck and thorax. Clinical features vary widely, involvement range from typical nail changes only to generalized disease. The disease runs a chronic relapsing course that persists throughout life, although it don’t affect general health much. Exacerbations of Darier’s disease can occur from exposure to heat, sunlight, trauma, UVB and during menstruation. Histology shows dyskeratosis in spinous layer (corps ronds) and stratum corneum (grains), suprabasal acantholysis and clefts. Basic measures to manage Darier’s disease may include using sunscreen, avoiding hot environments. Moisturizers with urea or lactic acid can reduce scaling of the skin. A mild to moderate potency topical steroid and topical antibiotics are often useful for inflammation. Topical medications may include topical retinoids. Oral retinoids is the most effective medical treatment for Darier’s disease. Other treatments may include oral antibiotics to clear bacterial infection, oral acyclovir to treat or suppress herpes simplex virus infection; dermabrasion; electrosurgery. We report a 15 years old female having malodourous greyish colored warty plaques in sebaceous distribution areas for three years. There was no mucosal involvement. Skin biopsy revealed abnormal formation of keratin tissue and acantholysis. Topical retinoid adapalene was prescribed along with topical 10% urea cream. Advice given to improve general hygiene, preference of cotton clothes, avoiding heat and sunlight.