Cutaneous manifestations of cystic fibrosis - 20/08/24
Abstract |
Cystic fibrosis (CF) is caused by a mutation in the Cystic fibrosis transmembrane conductance regulator (CFTR) gene, and features recurrent sinus and pulmonary infections, steatorrhea, and malnutrition. CF is associated with diverse cutaneous manifestations, including transient reactive papulotranslucent acrokeratoderma of the palms, nutrient deficiency dermatoses, and vasculitis. Rarely these are presenting symptoms of CF, prior to pulmonary or gastrointestinal sequelae. Cutaneous drug eruptions are also highly common in patients with CF (PwCF) given frequent antibiotic exposure. Finally, CFTR modulating therapy, which has revolutionized CF management, is associated with cutaneous side effects ranging from acute urticaria to toxic epidermal necrolysis. Recognition of dermatologic clinical manifestations of CF is important to appropriately care for PwCF. Dermatologists may play a significant role in the diagnosis and management of CF and associated skin complications.
Le texte complet de cet article est disponible en PDF.Key words : adverse events, transient reactive papulotranslucent acrokeratoderma of the palm, CFTR, Cystic fibrosis, nutrient deficiency dermatitis, vasculitis
Abbreviations used : ANCA, APK, BPI-ANCA, CF, CFNDD, CFTR, EFAs, PwCF
Plan
Funding sources: None. |
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Patient consent: Not applicable. |
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IRB approval status: Not applicable. |
Vol 91 - N° 3
P. 490-498 - septembre 2024 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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