LEUKODERMIC MACULES IN KERATOSIS FOLLICULARIS (DARIER'S DISEASE)

Four black African patients, two men and two women, were seen. Patient 1 was a Dinka from the southern Sudan studied in 1981. Patients 2, 3, and 4 were seen in the period 1986 to 1990 in South Africa. On examination all four patients had typical lesions of keratosis follicularis consisting of firm, greasy, crusted keratotic papules in the seborrheic areas of the trunk and face, with coalescing lesions on the scalp. The manifestations of keratosis follicularis differed in extent and severity. Acrokeratosis verruciformis-iike lesions on the shins and nail changes were observed in ail four patients. Involvement of the volar aspects of the hands and feet was variable, ranging from none, through delicate pits, to severely disabling plantar keratoderma. The oral mucosa was involved in only one patient. All four patients had widespread guttate markedly hypopigmented macules most prominent on the abdomen and chest (Figs. 1 and 2). In two patients the anterior aspects of the thighs and legs were also involved. The macules ranged from 1 to 4 mm in diameter and were distributed mainly in a perifoUicular pattern. In three patients the macules were discrete, while in one they coalesced to form thin reticulate strands. The leukodermic macules occurred in areas where classic papules of keratosis follicularis were absent or very sparse. They had not been preceded by papular keratotic lesions and appeared to evolve separately from them. During a 5year follow-up of patient 2, we observed the development of keratotic papules in some areas previously affected only by leukodermic macules. They erupted selectively between the macular elements.