C.Y. Chew, R. Nguyen
Darier’s disease is a dominantly inherited acantholytic der- matosis which typically occurs in childhood and adoles- cence in patients with a positive family history. Sporadic cases and late onset cases are rarely reported in the litera- ture. We present the case of a 75-year-old female who pre- sents with sporadic late onset Darier’s Disease as well as a review of the literature. Histopathology showed hyperker- atosis, parakeratosis, papillomatosis and elongation of rete ridges in the epidermis, as well as suprabasal acantholysis. Dyskeratotic cells (corps ronds) were present in the upper malpighian layer, consistent with Darier’s disease. The patient was treated effectively with acitretin 10 mg per day, topical betamethasone dipropionate 0.05% ointment, soap free wash and Sorbolene moisturiser. Our atypical case highlights the potential for Darier’s Disease, a condi- tion which most commonly presents in early life, to spo- radically arise in the elderly. Dermatologists should consider Darier’s Disease as a potential differential diagno- sis in elderly patients with classical clinical signs.