Darmstadt G L, McGuire J, Ziboh V A
Department of Pediatrics, Children's Hospital and Regional Medical Center, Seattle, Washington 98105, USA.
Pediatr Dermatol. 2000 Sep-Oct;17(5):337-47. doi: 10.1046/j.1525-1470.2000.017005337.x.
Rash is a rare presenting sign of cystic fibrosis (CF) complicated by protein-calorie malnutrition. We measured essential fatty acid (EFA) levels in the serum of a 4-month-old girl with an erythematous, desquamating, periorificially accentuated rash in association with malnutrition and her 2-year-old sister who was diagnosed concurrently with CF but had no rash or signs of malnutrition. Both patients had biochemical evidence of EFA deficiency, suggesting that development of the rash is multifactorial. Clinical presentation, management, and possible modes of pathogenesis of the rash are reviewed. Pathogenesis of the rash appears to involve a complex interaction among deficiencies of EFAs, zinc, protein, and possibly copper, leading to disordered prostaglandin metabolism or cytokine production, or free radical-induced damage to cellular membranes due to a lack of nutrient-derived protective antioxidants.
