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Shwachman Syndrome: A Case Report
Author(s) -
Mortureux Patricia,
Taïeb Alain,
Bazeille JeanEtienne Surlève,
Hehunstre Jean Pierre,
Maleville Jean
Publication year - 1992
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1992.tb00327.x
Subject(s) - medicine , pediatrics
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 liposluble vitamins, and suggested a causative link with the skin lesions.