Pityriasis Rubra Pilaris of Familial Type

The superficial clinical resemblance of certain cutaneous diseases to the phrynoderma of clinical and experimental vitamin A deficiency has led to a reconsideration of such conditions as possible manifestations of avitaminosis A. Among them are keratosis pilaris1(ichthyosis follicularis, lichen pilaris, etc.), Darier's disease2(keratosis follicularis) and pityriasis rubra pilaris.3 Lehman and Rapaport1studied a group of 9 children who exhibited the lesions of keratosis pilaris. They observed that the photometric values for dark adaptation as determined by the biophotometer were abnormal for these patients and that both this defect and the cutaneous lesions were for the most part corrected by administration of vitamin A. They concluded that keratosis pilaris and its synonyms were manifestations of vitamin A deficiency. Peck, Chargin and Sobotka2successfully treated Darier's disease with vitamin A and interpreted this condition as a "physiologic nevus with a hereditary weakness in vitamin A