KERATOSIS FOLLICULARIS-SPINULOSA-DECALVAN S - THERAPY WITH ORAL RETINOIDS IN THE INFLAMMATORY STAGE

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked d isorder of keratinization of the hair follicle associated with corneal dystrophy. The clinical picture is characterized by solid follicular hyperkeratosis, especially on the exposed skin, sparse eyebrows/eyelas hes, follicular scaling and scarring alopecia of the scalp, dry skin a nd ocular symptoms with keratitis and photophobia. We describe the thr ee stages of the disease: onset, inflammation and partial remission an d the treatment appropriate in each. Two patients in the inflammatory stage of KFSD, with recurrent deep, fibrosing folliculitis and perifol liculitis followed by spreading and scarring alopecia on the scalp, re sponded to oral therapy with retinoids. In both cases there was a dist inct and lasting remission of the inflammation and stabilization of th e spreading alopecia after treatment with etretinate (Tigason), up to 0.8 mg/kg body weight, or isotretinoin (Roaccutan), 0.5 mg/kg body wei ght, for 12 weeks. The follicular spinulous hyperkeratosis became soft er, but persisted. Thus, oral therapy with retinoids appears helpful i n the inflammatory stage of KFSD, even though there is little improvem ent in the follicular hyperkeratosis.