Wenk Kurt S, Higgins Kristen B, Greer Kenneth E
Department of Dermatology, University of Virginia Health System, Charlottesville, USA.
Arch Dermatol. 2010 Feb;146(2):171-4. doi: 10.1001/archdermatol.2009.361.
Patients with cystic fibrosis classically present with evidence of pulmonary disease, exocrine pancreatic insufficiency, and high sweat chloride concentrations. Dermatitis as an initial manifestation of the disease is uncommon and has been attributed to multiple nutritional deficiencies.
We describe the case of a 3-month-old female infant with cystic fibrosis presenting with dermatitis in the setting of protein-energy malnutrition. A review of the laboratory study results in this case and others showed that a deficiency in zinc, essential fatty acids, and protein likely contributes to the development of the rash seen in cystic fibrosis.
Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with protein-energy malnutrition in these patients, it is important to consider cystic fibrosis as a possible diagnosis in any infant presenting with a rash and other signs of malnutrition. The relative contribution of specific nutritional deficiencies and the degree to which they influence and interact with each other in producing the dermatitis remain unclear, although they may all affect a common underlying metabolic pathway.
囊性纤维化患者通常表现出肺部疾病、外分泌胰腺功能不全以及高汗液氯化物浓度的证据。皮炎作为该疾病的初始表现并不常见，且被认为与多种营养缺乏有关。
我们描述了一名3个月大的患有囊性纤维化的女婴病例，该婴儿在蛋白质-能量营养不良的情况下出现了皮炎。对该病例及其他病例的实验室研究结果进行回顾发现，锌、必需脂肪酸和蛋白质的缺乏可能导致了囊性纤维化患者皮疹的出现。
鉴于这些患者诊断时常出现延迟，以及与蛋白质-能量营养不良相关的发病率和死亡率增加，对于任何出现皮疹和其他营养不良迹象的婴儿，将囊性纤维化视为可能的诊断很重要。尽管特定营养缺乏的相对贡献以及它们在产生皮炎过程中相互影响和相互作用的程度尚不清楚，但它们可能都影响一个共同的潜在代谢途径。
