弥漫性皮炎：囊性纤维化一种意想不到的初始表现。

Diffuse dermatitis: An unexpected initial presentation of cystic fibrosis.

作者信息

Lovett Audrey, Kokta Victor, Maari Catherine

机构信息

Division of Pediatric Dermatology, Ste-Justine Hospital, University of Montreal Hospital Center, Montreal, Quebec, Canada.

出版信息

J Am Acad Dermatol. 2008 Feb;58(2 Suppl):S1-4. doi: 10.1016/j.jaad.2006.04.012.

DOI:10.1016/j.jaad.2006.04.012

PMID:18191689

Abstract

Cystic fibrosis is an autosomal recessive disease that typically presents with pulmonary symptoms. Diffuse rash related to protein energy malnutrition can rarely be a presenting sign of cystic fibrosis in infancy. We report such a case and relate the difficulties of establishing a diagnosis. We also discuss possible pathophysiological mechanisms, histopathology, prognosis, and treatment.

摘要

囊性纤维化是一种常染色体隐性疾病，通常表现为肺部症状。与蛋白质能量营养不良相关的弥漫性皮疹在婴儿期很少会是囊性纤维化的首发体征。我们报告了这样一例病例，并阐述了确诊的困难之处。我们还讨论了可能的病理生理机制、组织病理学、预后及治疗方法。