ANCA associated vasculitis with skin ulcers of the cald, clinically manifested as pyoderma gangrenosum
Authors:
B. Průchová 1; H. Štilcová 2; M. Podhola 3; D. Kacerovská 4; T. Soukup 2
Authors‘ workplace:
Oblastní nemocnice Náchod a. s., Interní oddělení Nemocnice Rychnov nad Kněžnou
1; Subkatedra revmatologie, II. interní gastroenterologická klinika LF UK a FN, Hradec Králové
2; Fingerlandův ústav patologie LF a FN, Hradec Králové
3; Šiklův ústav patologie LF a FN, Plzeň
4
Published in:
Čes. Revmatol., 29, 2021, No. 4, p. 228-236.
Category:
Case Report
Overview
ANCA (antineutrophil cytoplasmic antibody) – associated vasculitis (AAV) is one of the chronic systemic inflammatory diseases of small and medium caliber vessels. The most commonly affected are the airways, where necrotizing granulomas form, and the kidneys in the form of focal segmental necrotizing glomerulonephritis with crescent formation. Diagnosis is based on clinical symptoms, detection of ANCA antibodies, and biopsy of the affected organ tissue. Pyoderma gangrenosum (PG) is a very rare chronic inflammatory skin disease with the formation of painful ulcers. The etiopathogenesis is not fully understood; it is probably immunopathology with neutrophil dysfunction. We determine the diagnosis on the basis of diagnostic criteria, which are based primarily on histopathological findings, a clinical picture with a rapid response to immunosuppressive therapy. In ⅔ of cases, it is associated with other systemic autoimmune diseases (inflammatory bowel diseases, arthritis, hematological malignancies, etc.)
We describe a case of a hitherto healthy 53-year-old woman who developed painful ulcerative defects of the right calf, followed by the development of phlegmon. Defects developed after a minor injury. Doppler ultrasonography of the arteries excluded the ischemic basis of the resulting defect, and the culture excluded the infectious etiology of the disease. Subsequent skin biopsy from a defect with numerous neutrophilic granulocytes found a diagnosis of PG. The diagnosis of ANCA vasculitis was made on the basis of positive ANCA antibodies (against type 3proteinase) and histological findings from a kidney biopsy.
The diagnosis of PG is rare, so it is necessary to think about it in non-healing ulcers. If the diagnosis is made, it is important to look for possible associated diseases. As in most cases, an association with other serious conditions has been demonstrated; in our case, ANCA necrotizing small-vessel vasculitis was found.
Keywords:
ANCA associated vasculitis – pyoderma gangrenosum – shin ulcers
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