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Shwachman syndrome: a case report
P Mortureux1, A Taïeb, J E Bazeille
1Services de Dermatologie et Pédiatrie A, Hôpital des Enfants, Bordeaux, France.
Pediatric Dermatology
|March 1, 1992
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.