皮膚炎ジャーナル

概要

Cutaneous Vasculitis Associated to Cystic Fibrosis

Ruiz Beguerie J and Fernandez J

Cutaneous vasculitis is a rare complication of cystic fibrosis. The exact incidence is unknown because there are only isolated cases reported. It usually affects patients with severe lung disease and it is believed to be associated with a worse prognosis. While the skin is the most frequently affected site, vasculitis may involve other organs. We present a patient diagnosed with cystic fibrosis since childhood, with a history of liver transplantation and controlled lung disease. At the age of 18 years, the patient consulted with recurrent episodes of cutaneous vasculitis. Three years later, associated with an increase in skin rash, he evolved with severe respiratory failure and cardiopulmonary transplant requirement.