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.
MeSH terms
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Bone Marrow Diseases / complications
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Bone Marrow Diseases / diagnosis*
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Child
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Child Nutrition Disorders / complications
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Child Nutrition Disorders / diagnosis*
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Child Nutrition Disorders / metabolism
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Developmental Disabilities / complications
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Developmental Disabilities / diagnosis*
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Exocrine Pancreatic Insufficiency / complications
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Exocrine Pancreatic Insufficiency / diagnosis*
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Humans
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Male
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Neutropenia / complications
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Neutropenia / diagnosis*
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Skin Diseases / etiology*
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Syndrome