While the pulmonary and pancreatic involvement of cystic fibrosis (CF) is commonly described and therefore best studied, the cutaneous manifestations are frequently underdiagnosed, despite being important markers of disease severity. We report a case of antineutrophil cytoplasmic antibody-negative cutaneous vasculitis in a 15-year-old female CF patient in tandem with infection and subsequent colonization by Burkholderia cepacia complex (BCC). The flares of cutaneous vasculitis is associated closely with an infective exacerbation of CF and improved upon treatment of the infective exacerbation. We further discuss how the appearance of BCC colonization and cutaneous vasculitis affected both lung function and lung parenchyma by tracking spirometry and imaging changes over the subsequent four years.
Keywords: burkholderia cepacia complex; cutaneous vasculitis; cystic fibrosis.
Copyright © 2020, Klimko et al.