title: [Keratosis follicularis decalvans: nosological discussion of Siemens' disease. Apropos of 3 cases].
abstract: We describe 3 cases of keratosis follicularis decalvans (Siemens' disease): a 15 year old boy and a 7 year old boy and his father. They represent 2 different patterns of the disease with different clinical courses and genetic background: an autosomal dominant type of good prognosis with elevated argininemia and absence of follicular atrophy in both father and son, a sporadic type, clinically severe, with follicular atrophy. This raises the question of the nosology of the so-called Siemens' disease, since it includes actually different diseases of variable prognosis.
authors.affiliation: Service de dermatologie, CHU Morvan, Brest, France.
authors.forename: G
authors.initials: G
authors.lastname: Guillet
authors.forename: F
authors.initials: F
authors.lastname: Labouche
authors.forename: F
authors.initials: F
authors.lastname: Cambazard
authors.forename: P
authors.initials: P
authors.lastname: Plantin
authors.forename: Y
authors.initials: Y
authors.lastname: Gall
authors.forename: A
authors.initials: A
authors.lastname: Le Jollec
authors.forename: A
authors.initials: A
authors.lastname: Zagnoli
authors.forename: P
authors.initials: P
authors.lastname: Parent
mesh_terms: D000293:Adolescent; D000328:Adult; D000505:Alopecia / Q000151:congenital*; D002648:Child; D007644:Darier Disease / Q000145:classification* / Q000235:genetics; D006801:Humans; D008297:Male; D013997:Time Factors
vernacular_title: Kératoses folliculaires décalvantes: discussion nosologique de la maladie de Siemens. A propos de trois cas.