Cornifying Darier's disease

A 48-year-old Caucasian man recounted the onset of keratotic papules on the trunk at the age of 8 years, with subsequent spread to the forearms, scalp , and forehead. His most severe disease was present on the legs. He complai ned of pain, itching, and noted exacerbations in the summer and with sweati ng. The family history was negative. On physical examination, the most striking finding was that of extensive, m arkedly hyperkeratotic plaques on the lower legs > (Fig. 1). His scalp, for ehead, chest, and back exhibited mild involvement, with scattered brown ker atotic papules, while his forearms showed mildly hyperkeratotic plaques. Fl at-topped brown papules were present on the dorsum of the hands, with a few keratotic papules on the palms, and a few nails with distal notching and r ed longitudinal streaks. There were no palmar pits or oral mucosal lesions. A shave biopsy was performed of a plaque on the leg, and showed a papilloma tous and markedly hyperkeratotic lesion > (Fig. 2). Suprabasal acantholysis in the elongated rete produced characteristic lacunae. The acantholysis wa s associated with dyskeratosis including corps ronds and grains > (Fig. 3). Together, these features were characteristic of Darier's disease. Treatment years earlier with topical retinoids, topical steroids, topical k eratolytics, and multiple oral antimicrobials had been unsuccessful, and is otretinoin had been discontinued due to elevated triglycerides. Treatment w as initiated with acitretin and, after 3 months, mild improvement was noted .