Cutaneous manifestations of Wegener's granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status
| Autor: | Lawrence E. Gibson, Nneka I. Comfere, Nada C Macaron |
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| Rok vydání: | 2007 |
| Předmět: |
Adult
Male Systemic disease Pathology medicine.medical_specialty Histology Adolescent Myeloblastin Dermatology Disease urologic and male genital diseases Skin Diseases Pathology and Forensic Medicine Antibodies Antineutrophil Cytoplasmic immune system diseases Proteinase 3 Medicine Humans Serologic Tests cardiovascular diseases Seroconversion skin and connective tissue diseases Child Anti-neutrophil cytoplasmic antibody Aged Retrospective Studies Skin Inflammation Granuloma business.industry Vascular disease Granulomatosis with Polyangiitis Middle Aged medicine.disease respiratory tract diseases Vasculitis Leukocytoclastic Cutaneous Female business Vasculitis Systemic vasculitis |
| Zdroj: | Journal of cutaneous pathology. 34(10) |
| ISSN: | 0303-6987 |
| Popis: | Background: Wegener’s granulomatosis (WG), a systemic vasculitis, can be associated with cutaneous signs and symptoms before, during or after the diagnosis of systemic disease. Methods: We reviewed clinical and histologic features of cutaneous lesions from 17 patients with WG. The temporal relationship between development of cutaneous symptoms and onset of systemic disease was determined, and antineutrophil cytoplasmic antibody (ANCA) status of the patients was also established. Results: In six patients, systemic and cutaneous disease developed concurrently. In eight patients, cutaneous disease developed after patients received the diagnosis of systemic disease. In three patients, cutaneous disease preceded systemic disease. Cytoplasmic ANCA or proteinase-3-ANCA [c-ANCA/proteinase 3 (PR3)-ANCA] serologic test results were negative for one patient when cutaneous disease developed, and one patient had c-ANCA/PR3-ANCA seroconversion a year before systemic disease developed. Histopathologic features of cutaneous WG were not limited to leukocytoclastic vasculitis; they also included acneiform perifollicular and dermal granulomatous inflammation and palisaded neutrophilic and granulomatous inflammation. Conclusions: Patients with WG can present initially with cutaneous symptoms. Histopathologic patterns vary, but leukocytoclastic vasculitis is most commonly noted. Patients with WG and skin lesions are likely to have positive c-ANCA/PR3-ANCA serologic test results. |
| Databáze: | OpenAIRE |
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