[Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage]

Abstract

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked disorder 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/eyelashes, follicular scaling and scarring alopecia of the scalp, dry skin and ocular symptoms with keratitis and photophobia. We describe the three stages of the disease: onset, inflammation and partial remission and the treatment appropriate in each. Two patients in the inflammatory stage of KFSD, with recurrent deep, fibrosing folliculitis and perifolliculitis followed by spreading and scarring alopecia on the scalp, responded to oral therapy with retinoids. In both cases there was a distinct and lasting remission of the inflammation and stabilization of the spreading alopecia after treatment with etretinate (Tigason), up to 0.8 mg/kg body weight, or isotretinoin (Roaccutan), 0.5 mg/kg body weight, for 12 weeks. The follicular spinulous hyperkeratosis became softer, but persisted. Thus, oral therapy with retinoids appears helpful in the inflammatory stage of KFSD, even though there is little improvement in the follicular hyperkeratosis.