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

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