A Case Report of Cystic Fibrosis Complicated by Burkholderia Cepacia and Cutaneous Vasculitis

Abstract

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.

A case of localized paranasal sinusitis associated with Burkholderia cenocepacia ST 1880 in a cystic fibrosis patient

“…The Bcc lesions in CF lungs are often combined with the presence of extrapulmonary Bcc loci. Bcc-associated sinusitis, cutaneous vasculitis, and bloodstream infections are known [ [12] , [13] , [14] ]. However, in almost all the described cases, extrapulmonary Bcc pathology was secondary and was associated with an unfavorable course of the lung disease.…”

Burkholderia cepacia complex in cystic fibrosis: critical gaps in diagnosis and therapy

“…The clinical manifestations of Bcc infection in people with CF are highly variable and unpredictable [ 4 , 45 ], although they are often associated with a more severe and rapid lung impact than that caused by other colonizing bacteria in the airways of CF patients, such as P. aeruginosa [ 2 , 22 , 24 , 38 ]. Bcc commonly cause exacerbated lung inflammation that dramatically worsens the lung function of CF patients [ 2 , 22 , 24 ]. In addition, they can cross the epithelial barrier, enter the bloodstream, and lead to bacteremia [ 45 ].…”

Overview of infections as an etiologic factor and complication in patients with vasculitides

“…In leukocytoclastic vasculitis, the presence of upper respiratory tract infections shortly before the development of vasculitis was more common than in those with IgA vasculitis [5]. Cutaneous vasculitis has been described in patients with cystic fibrosis due to respiratory infections due to Pseudomonas aeruginosa, S. aureus, Haemophilus influenza, and Burkholderia cepacia complex [8,31,32]. Furthermore, a cutaneous small-vessel vasculitis may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae [33].…”