Cystic fibrosis (CF) is an autosomal recessive disease that occurs in 1 in 2500 live births in Northern American and Northern European White populations. The disease results from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

CF nutrient deficiency dermatitis (CFNDD) is a consequence of CF-associated gastrointestinal tract dysregulation and the resultant malabsorption of specific nutrients, including zinc, essential fatty acids (EFAs), liposoluble vitamins, and protein. Consequently, CFNDD often presents with overlapping clinical features of these deficiencies. Systemic findings associated with CFNDD include failure to thrive, hepatomegaly, numerous large, green stools, and periorbital and extremity pitting edema. Infants with CFNDD often have a history of several large, loose stools per day occurring at the onset or shortly after the development of skin lesions. Alopecia, anemia, and hypoproteinemia may also be present in patients with protein deficiency.

Patients with untreated CF are at particularly increased risk of developing CFNDD. CFNDD often occurs in infancy at about 2 weeks to 6 months of age and, prior to the era of newborn screening, was occasionally the presenting sign of CF.

A few cases of the characteristic eruption of CFNDD in patients with episodic arthritis of CF have been described.