The results of the examination of three generations of a family with an unusually high incidence of Darier's disease are reported. Parallel with the cutaneous symptoms, mental deficiency and psychotic episodes were noted. All affected members showed nail changes in the form of longitudinal striation and nicking of the free edge of the nail plates, which was the most characteristic feature. Involvement of the mucous membranes of the mouth occurred in a large percentage of patients, and cytological smears showed typical cellular abnormalities. Forms frustes were observed in patients who exhibited clinically minimal and inconspicuous lesions, which, on histological examinations, showed the characteristic pathological changes of Darier's disease. Remissions could be induced by treatment with a water-soluble vitamin A preparation.
GETZLER NA, FLINT A. Keratosis Follicularis: A Study of One Family. Arch Dermatol. 1966;93(5):545–549. doi:10.1001/archderm.1966.01600230049015
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