Three family members, at initial evaluation, had generalized comedonal lesions with histologic changes of acantholysis and dyskeratosis. A total of nine cases of this entity, termed familial dyskeratotic comedones, have been documented in the literature. It appears to have autosomal dominant inheritance and onset in childhood or adolescence. Lesions are asymptomatic except for occasional pruritus or inflammation, and general health is undisturbed. A history of acne vulgaris is seen in four of nine patients and is the only associated skin disease. Treatment with oral isotretinoin produced no improvement in two patients. Electron microscopy revealed changes similar to those seen in Darier's disease.