Shwachman syndrome: a case report

Pediatr Dermatol. 1992 Mar;9(1):57-61. doi: 10.1111/j.1525-1470.1992.tb00327.x.

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.

Publication types

  • Case Reports

MeSH terms

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