Surgical treatment of Darier disease: a case report

Abstract

Darier disease (Keratosis folicullaris) was described independently by Darier and White in 1989. It is one of the most common genodermatoses, with an incidence of 1:30,000 – 100,000. This disease is a result of mutation in the ATP2A2 gene. The typical lesions are yellow – brown keratotic papules or plaques, usually found in the seborrheic areas such as scalp, forehead, retroauricular folds, neck, chest, axillae and extremities. Aim: The Aim is to evaluate the results of surgical treatment of Darier disease. Materials and Methods: A 41-year-old man presented with keratotic lesions on the feet and palms since 25 years. Patient was treated with emolliants, keratolytics (salicylic acid, urea), PUVA therapy, and systemic retinoids (isotretinoin), but treatment was without improvement. Examination revealed multiple keratotic yellow plaques on the palms. Exophytic keratotic warty texture papules were seen on the feet. Cysts and closed comedones were on the skin of face and chest. Surgical excision of keratotic masses surgical on feet was made. Firstly, partial excision of medial surface on the right sole on the level of healthy derma was made. The wounds were left for epithelization. Second keratotic masses excision was made after five months on the right sole and after six months on the left sole. Results: Re-ephitelialization on the excision site was observed and there weren’t evident signs of infection and recurrence. Conclusions: Surgical excision of keratotic masses can be successesful treatment of Darier disease for patients who don’t respond to topical and systemical treatment.