Cutaneous Symptoms of Cystic Fibrosis: A Narrative Review

Cystic fibrosis (CF) is caused by a mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene and is characterized by recurrent pulmonary infections, fat malabsorption, and malnutrition. CF is associated with diverse dermatologic symptoms, including aquagenic palmoplantar keratoderma, nutrient deficiency dermatitis, and vasculitis. Sporadically, these manifestations may represent the initial symptoms of cystic fibrosis. Drug-induced skin reactions are commonly observed in patients with cystic fibrosis (pwCF) due to the chronic use of antibiotics. Therapy with CFTR modulators is considered the gold standard in the treatment of cystic fibrosis. Nevertheless, they are associated with numerous side effects, ranging from rash to Stevens-Johnson syndrome. Identifying the cutaneous manifestations of cystic fibrosis may support comprehensive care for patients. In this regard, dermatologists may play an important role in the management of associated skin complications.