ABSTRACT
INTRODUCTION: Wegener's granulomatosis is a rare systemic vasculitis, characterized by involvement of the upper airways, lungs and kidneys; other organs may also be affected. Cutaneous lesions occur frequently during the disease course but seldom as its initial presentation. Digital necrosis and splinter hemorrhages of fingernails are described, but very rarely. CASE: We report the case of a 55-year-old man presenting multisystemic Wegener's granulomatosis with mucosal and cutaneous involvement at initial presentation: tongue and labial ulcers, digital necrosis, splinter hemorrhages of fingernails, and purpura. The PR3-ANCA titer was high. Imaging and the histological findings of both cutaneous and renal biopsies suggest a diagnosis of Wegener's granulomatosis. He was treated with prednisone, cyclophosphamide, and cotrimoxazole. Cutaneous and mucosal symptoms disappeared 6 weeks after treatment began. DISCUSSION: The particularity of this case is the association of four separate cutaneous manifestations of Wegener's granulomatosis - two of them very rare - as the initial presentation of this multisystemic disease.
Subject(s)
Granulomatosis with Polyangiitis/diagnosis , Nail Diseases/etiology , Oral Ulcer/etiology , Purpura/etiology , Fingers/pathology , Granulomatosis with Polyangiitis/complications , Hemorrhage/etiology , Humans , Male , Middle Aged , Necrosis/etiology , Skin/pathologyABSTRACT
INTRODUCTION: Several cases of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) have been reported in patients treated with synthetic anti-thyroid drugs but only 2 cases have incriminated benzylthiouracil. OBSERVATION: A 36 year-old woman, 3 years after treatment with benzylthiouracil, rapidly developed progressive kidney failure, related to a pauci-immune extra-capillary glomerular nephropathy and necrotic vasculitis lesions. The search for p-ANCA was positive with anti-myeloperoxidase specificity. She was treated with corticosteroids and 6 monthly intravenous pulses of cyclophosphamide substituted by azathioprine. Renal failure and proteinuria significantly improved. However the high level of p-ANCA. CONCLUSION: ANCA vascularities are a rare but serious complication of treatment with synthetic thiouracile-type anti-thyroid drugs. The ANCA must be measured when confronted with a systemic manifestation during treatment.
