Cutaneous Wegener's granulomatosis: a variant or atypical localized form?

摘要: A 74-year-old woman presented with an antineutrophil cytoplasmic antibody titre-negative, treatment-responsive Wegener's granulomatosis confined to the integument. She initially a painful left postauricular nodulo-ulcerative lesion chronically discharging sinuses. This was effectively treated short, 3-month course of cyclophosphamide and 24 months oral prednisone. After 5 in remission, she developed further similar ulcers, addition painless nodules on her ankles feet bilaterally. These lesions resolved extra 32 high-dose prednisone therapy before complete remission. At most recent review, there no evidence disease recurrence 21 after ceasing all active treatment. Histology demonstrated granulomatous inflammation. No systemic progression upper respiratory tract, lung or kidney detected. case highlights importance being aware atypical partial presentations granulomatosis. diagnosis needs be considered patients presenting culture-negative chronic ulcer, where malignancy trauma have been excluded. will avoid unnecessary surgery ensure early effective treatment that is disfiguring usually fatal if inappropriately treated.