Darier disease, also known as keratosis follicularis is a rare autosomal dominant genodermatosis, commonly presents with follicular and extrafollicular greasy hyperkeratotic papules and plaques, arising primarily in seborrheic areas. However, other uncommon clinical presentations have been reported. This paper reports a rare presentation of Darier's disease, which mainly showed comedonal papules. The patient is a 35 year-old male presented with 8 months history of mildly itchy slowly progressive generalized skin lesions. His past medical history, drug history and review of systems were all unremarkable. His parents are non-consanguineous and no similar case in the family. Skin examination revealed multiple tiny non-scaly brownish papules (comedonal like) scattered on his scalp, neck, upper extremities, abdominal wall, chest wall and back. His nails showed Longitudinal red and white streaks as well as notching of the free edge of the nail plate. Hairs, and mucus membranes were all normal. Skin biopsy showed Hyperkeratosis, acanthosis, epidermal acantholysis and suprabasal clefting. Based on the clinical and the histopathologic features, the diagnosis of Darier Disease was made.
