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一例罕见的肉芽肿性韦格纳氏病伴多发坏疽性脓皮病样溃疡。

A rare case of granulomatous Wegener with multiple pyoderma gangrenosum-like ulcers.

作者信息

Jorgaqi Ekleva, Fida Monika, Janushaj Elmijola, Mala Ritjana, Vasili Ermira

机构信息

Dermatology-Venereology Service, UHC "Mother Theresa", Tirana, Albania.

Dermatology-Venereology Department, Faculty of Medicine, UMT, Tirana, Albania.

出版信息

Dermatol Ther. 2020 Mar;33(2):e13255. doi: 10.1111/dth.13255. Epub 2020 Feb 17.

Abstract

Wegener's granulomatosis, is a rare form of systemic vasculitis characterized by necrotic granulomas around the blood vessels. We report a case of a healthy 43-year-old man presented with multiple ulcerated lesions in head and all over the body, that has appeared spontaneously. It was characterized by a giant deep ulceration covered by thick purulent, in size from 3 to 4 cm, deep; sub febrile temperature, and minimal pain associated. Skin biopsies results pyoderma gangrenosum (PG)-like features: anti-neutrophil cytoplasmic antibody (cANCA) positive. Six months later, the patient developed dispea episodes. CT scan (CT) result with multiple focal opacities. He was diagnosed with wegener granulomatosis (WG) and was treated with corticosteroids and ciclophosphamide. Skin involvement in WG is not common and lesions in the skin vary from purpura, paniculitis to ulceration. In our case, skin elements are the first manifestations. Whether these elements are Wegener manifestation or we have coexistence of PG with WG, it remains a still uncertain question to answer. There is no histopathologic pathognomic elements for both. Skin involvement in WG with PG-like lesions have been rarely reported. In front of this type of widespread ulcerations, resistant to treatment we should be aware that one of the reasons may be a disease of autoimmune etiology such as WG.

摘要

韦格纳肉芽肿病是一种罕见的系统性血管炎,其特征为血管周围出现坏死性肉芽肿。我们报告一例43岁健康男性病例,该患者头部和全身出现多处自发出现的溃疡性病变。其特征为一个巨大的深部溃疡,表面覆盖着厚厚的脓性分泌物,大小为3至4厘米,较深;低热,伴有轻微疼痛。皮肤活检结果显示具有坏疽性脓皮病(PG)样特征:抗中性粒细胞胞浆抗体(cANCA)呈阳性。六个月后,患者出现呼吸困难发作。CT扫描结果显示有多个局灶性混浊。他被诊断为韦格纳肉芽肿病(WG),并接受了皮质类固醇和环磷酰胺治疗。WG累及皮肤并不常见,皮肤病变从紫癜、脂膜炎到溃疡不等。在我们的病例中,皮肤症状是首发表现。这些症状究竟是韦格纳病的表现,还是PG与WG并存,仍是一个有待解答的不确定问题。两者均无组织病理学特征性表现。伴有PG样病变的WG累及皮肤的情况鲜有报道。面对这种广泛的、对治疗有抵抗性的溃疡,我们应该意识到其中一个原因可能是自身免疫性病因的疾病,如WG。

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