Dermatitis as a Presenting Sign of Cystic Fibrosis

Three percent to 13% of cystic fibrosis patients presented with protein-energy malnutrition, characterized by hypoproteinemia, edema, and anemia, leading to high morbidity and mortality. This study described five patients who exhibited failure to thrive, hypoproteinemia, edema, and a cutaneous eruption before pulmonary symptoms and cystic fibrosis diagnosis. The rash, primarily on the extremities, perineum, and periorificial surfaces, developed into extensive, desquamating plaques within months. Alopecia was variable, and mucous membrane or nail involvement was absent. The rash resolved within 10 days of pancreatic enzyme and nutritional supplementation. The rash likely resulted from deficiencies in zinc, protein, and essential fatty acids, possibly mediated by altered prostaglandin metabolism. Recognizing this rash as a sign of cystic fibrosis could lead to earlier diagnosis and treatment, improving patient outcomes.