A rare case of vasculitis in cystic fibrosis: A clinical case

Respir Med Case Rep. 2023 Jan 27:42:101816. doi: 10.1016/j.rmcr.2023.101816. eCollection 2023.

Abstract

Introduction: Cystic fibrosis is known to cause serious complications, such as recurrent pulmonary infections, pancreatic insufficiency, and other symptoms related to exocrine gland dysfunction. A rare manifestation of the disease is discussed in this case of a 24-year-old female diagnosed with cystic fibrosis, a purpuric rash was documented during pulmonary infection flares. Skin biopsy shows a leukocytoclastic vasculitis eruption along with infection. Treatment options are limited and not well established. Our patient received a treatment based on colchicine 1mg per day with a total response. The patient was observed during two consecutive pulmonary infection flares separated by a few months, and a total remission without recurrence was found.

Conclusion: Considering its efficacy and safety, further scientific research about colchicine and vasculitis in cystic fibrosis should be aimed at in order to define a strong consensus between the disease and this treatment option.

Keywords: Bronchiectasis; CFTR, Cystic fibrosis transmembrane conductance regulator; Colchicine; Cystic fibrosis; Leukocytoclastic vasculitis; Purpuric rash.

Publication types

  • Case Reports