Keratosis Follicularis and Familial Benign Pemphigus in the Same Patient / 대한피부과학회지

ABSTRACT

Keratosis follicularis and familial benign pemphigus is an autosomal dominant inherited disease. Keratosis follicularis is characterized by keratotic papules predominantly on the seborrheic areas and familial benign pemphigus, recurrent bullous and vesicular dermatitis on the opposing surfaces. The differential diagnosis of the two diseases with histopathological findings is difficult and still in confusion. We present a case of a 23-year-old woman who had plaques composed of keratotic papules on the anterior shin areas and oozing crusted patches on the buttocks and calves which had some resemblance to the clinical features of familial benign pemphigus. The histological features of the former were consistent with keratosis follicularis, whereas that of the latter was characteristic of familial benign pernphigus.