Protracted superficial Wegener's granulomatosis.
A 27-year-old woman presented with a right infra-auricular noduloulcerative lesion progressing to a peri-auricular pyoderma gangrenosum-like ulcer with destruction of her right earlobe over an 8-month period. Similar nodules appeared on the right malar and left infra-auricular regions. The cutaneous manifestations were associated with nasal congestion, rhinorrhoea and serosanguineous nasal crusting. Skin biopsy demonstrated suppurative granulomatous inflammation. Investigation of both renal and pulmonary function showed no abnormality. Serological testing revealed a positive cytoplasmic pattern antineutrophil cytoplasmic antibody with high proteinase-3 specificity, which in conjunction with the clinical findings is consistent with a diagnosis of protracted superficial Wegener's granulomatosis. Initial treatment with prednisone 1 mg/kg/day and azathioprine 100 mg/day resulted in complete resolution of her lesions. Reduction of the corticosteroid dose below 0.3 mg/kg/day led to recrudescence of cutaneous and upper respiratory tract symptoms, at which stage methotrexate was substituted for azathioprine with rapid induction of remission and further prednisone withdrawal. Thirty-two months after the initial diagnosis the patient remains well with no other organ involvement.
Sinovich
Vania
V
Department of Dermatology, Green Lane Hospital, Auckland, New Zealand. drsvafalk@hotmail.com
Snow
John
J
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001379:Azathioprine / Q000627:therapeutic use; D001706:Biopsy; D004359:Drug Therapy, Combination; D004426:Ear Deformities, Acquired / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D004431:Ear, External / Q000002:abnormalities; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D001379:Azathioprine; D011241:Prednisone