title: [Siemens I syndrome--a case history].
abstract: Report on a patient suffering from keratosis follicularis spinulosa decalvans (Siemens I syndrome). This ia a very rare clinical picture, at least in its complete form. The patient's father and brother are suffering from an abortive Siemens I syndrome. The condition is a form of hereditary parakeratosis, a spinulose keratosis with typical localization in the eyes and on the skin. Causal therapy is not possible; attempts at conservative and surgical treatment are described. Prognostically, the condition will probably come to a standstill at puberty. The description of this case is intended to illustrate the necessity of close cooperation between dermatologist and ophthalmologist.
mesh_terms: D000293:Adolescent; D000328:Adult; D007644:Darier Disease / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D005128:Eye Diseases / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D013577:Syndrome
vernacular_title: Beitrag zum Siemens-I-Syndrom.