Dermatitis as a Presenting Sign of Cystic Fibrosis

The document described five patients with cystic fibrosis who initially presented with dermatitis characterized by erythematous, scaling papules that progressed to desquamating plaques, primarily on the extremities, perineum, and periorificial surfaces. These skin manifestations appeared before pulmonary symptoms and the diagnosis of cystic fibrosis. The dermatitis was associated with protein-energy malnutrition, including hypoproteinemia, edema, and anemia, and resolved within 10 days of pancreatic enzyme and nutritional supplementation. The study suggested that cystic fibrosis should be considered in the differential diagnosis of infants with red, scaly rashes, especially when accompanied by failure to thrive and malnutrition, to enable earlier diagnosis and treatment.