[Severe toe gangrene as an early manifestation of Wegener granulomatosis in a young patient].
Gangrene of digits is a very rare manifestation of Wegener's granulomatosis (WG). We report a case of a 29-year-old woman with nonspecific symptoms like fever, weight loss, arthralgia, arthritis and high systemic inflammatory signs. On the grounds of a presumed infection the patient was treated with antibiotics which showed no effect. Within short she complained of pain and paresthesia of the right foot with a rapid lividity. Angiography demonstrated multiples stenoses and multisegmental occlusions of the lower leg arteries. Together with renal and pulmonal symptoms a WG was suspected, the diagnosis being confirmed by kidney biopsy and positive cANCAs. A few months after starting a combined therapy with cyclophosphamide and corticosteroids the patient showed a partial remission with a residual toe gangrene. Comparing the five worldwide reported cases with digital gangrene and our presentation there is a concordance of all in the occurrence of an extremely high disease activity together with a glomerulonephritis. The findings of p-ANCAs in our patient and positive Hbs antigen in another case of WG with digital gangrene suggests a relation to panarteriitis nodosa, where gangren is more common.
Binder
C
C
Rheuma-Einheit und Medizinische Poliklinik der LMU München.
Schattenkirchner
M
M
Krüger
K
K
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D005260:Female; D005734:Gangrene; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis*; D006801:Humans; D010488:Polyarteritis Nodosa / Q000175:diagnosis; D014034:Toes / Q000473:pathology*
D019268:Antibodies, Antineutrophil Cytoplasmic
Schwere Zehengangrän als frühe Manifestation der Wegener-Granulomatose bei einer jungen Patientin.