We report herein two cases of keratosis follicularis spinulosa decalvans (KFSD) and review the literature on this condition. The entity is one of a group of related disorders that shows keratosis pilaris with inflammation followed by atrophy. The clinical features and course of KFSD are characteristic. During infancy, keratosis pilaris begins on the face and, by childhood, progresses to involve the trunk and extremities. Sometime during childhood or up to the early teenage years, a cicatricial alopecia of the scalp and eyebrows develops and is the hallmark of this disorder. Hyperkeratosis of the palms and soles is a frequently associated finding and is usually manifested during adolescence. Other features occurring with this syndrome include atopy, photophobia, and corneal abnormalities. Sex-linked inheritance has been proposed by several authors.

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Using mitomycin C treated 3T3-Swiss fibroblasts as feeder cells, human keratinocytes derived from infant foreskins were subcultured in the presence of trans-retinoic acid (RA) and other retinoids. At 1 microgram/ml (3 x 10(-6) M) and higher RA concentrations plating efficiency was markedly reduced. Addition of retinoids to 1 microgram/ml after colonies were established produced no change in the rate of cell production, but caused differentiated cells to be sloughed earlier than in control cultures. Electron microscopy showed wider extra cellular spaces the contained numerous villi, increased numbers of microvilli at the surfaces of the outermost cells, and decreased number of cell layers all of which were consistent with the observed desquamatory effects of RA. Retinoic acid also extended the time during which cell population increased so that RA treated cultures produced more cells than control cultures over their respective lifetimes. Keratin polypeptides represented a smaller percentage of the total solubilizable protein and more keratin was present as acid soluble prekeratin in postconfluent RA treated cultures. This may be a consequence of early desquamation rather than a decrease in keratin synthesis. No unusual proteins were visible in RA treated cultures by simple sodium dodecylsulfate electrophoresis, nor was there increase in specific activities of three lysosomal enzymes.