title: Shwachman syndrome: a case report.
abstract: Cutaneous involvement is frequent in Shwachman syndrome, and includes various degrees of dry skin, and eczematous and ichthyosiform lesions. A 12-year-old boy with Shwachman syndrome had cutaneous involvement characterized by dry skin, perioral dermatitis, and follicular keratosis. Polymorphonuclear motility was decreased. A nutrition work-up showed a decrease in liposoluble vitamins, and suggested a causative link with the skin lesions.
authors.affiliation: Services de Dermatologie et Pédiatrie A, Hôpital des Enfants, Bordeaux, France.
authors.forename: P
authors.initials: P
authors.lastname: Mortureux
authors.forename: A
authors.initials: A
authors.lastname: Taïeb
authors.forename: J E
authors.initials: JE
authors.lastname: Bazeille
authors.forename: J P
authors.initials: JP
authors.lastname: Hehunstre
authors.forename: J
authors.initials: J
authors.lastname: Maleville
mesh_terms: D001855:Bone Marrow Diseases / Q000150:complications / Q000175:diagnosis*; D002648:Child; D015362:Child Nutrition Disorders / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism; D002658:Developmental Disabilities / Q000150:complications / Q000175:diagnosis*; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000175:diagnosis*; D012871:Skin Diseases / Q000209:etiology*; D013577:Syndrome