Wegener's granulomatosis in two sisters.
We describe two sisters with necrotising granulomas. The intervals between the onset of symptoms and diagnosis were eight and nine years. The pathological findings showed necrotising granulomas in both patients, this being consistent with a diagnosis of pathergic (Wegener's) granulomatosis. The one patient that accepted treatment responded well to cyclophosphamide, though the facial lesions responded only to radiation therapy. Since the second was almost asymptomatic we conclude that relatives of patients with Wegener's disease should be investigated.
D000328:Adult; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000235:genetics* / Q000473:pathology; D006801:Humans; D009297:Nasal Mucosa / Q000473:pathology; D012867:Skin / Q000473:pathology