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无症状乙肝病毒携带者合并潜在皮下坏死性静脉炎的类脂质渐进性坏死性血管病:类脂质渐进性坏死性血管病是真正的非血管炎性疾病吗?

Livedoid vasculopathy with underlying subcutaneous necrotizing venulitis in an asymptomatic hepatitis B virus carrier: is livedoid vasculopathy a true nonvasculitic disorder?

作者信息

Ishibashi Masafumi, Miyamoto Juria, Nagasaka Takeshi, Chen Ko-Ron

机构信息

Department of Dermatology, Saiseikai Central Hospital, Tokyo, Japan.

出版信息

Am J Dermatopathol. 2009 May;31(3):293-6. doi: 10.1097/DAD.0b013e31819560aa.

DOI:10.1097/DAD.0b013e31819560aa
PMID:19384073
Abstract

Livedoid vasculopathy has been accepted as a nonvasculitic disorder, but authentic vasculitis in the underlying subcutis can occur in cases of collagen disease and polyarteritis nodosa. We report a case of livedoid vasculopathy with underlying subcutaneous necrotizing venulitis in a 42-year-old carrier of hepatitis B virus. The patient also had a 15-year history of ankylosing spondylitis that was currently in remission. Skin lesions revealed superficial ulceration, purpura, atrophie blanche, and reticulate erythema on the lower extremities, and a skin biopsy showed a minimal dermal perivascular lymphocytic infiltrate with marked fibrin thrombi and fibrin deposits along luminal vessel walls, consistent with features of livedoid vasculopathy. However, necrotizing venulitis characterized by a predominant lymphocytic infiltrate in and around the vessel wall with marked fibrinoid vessel wall necrosis was found in the underlying subcutaneous tissue. A direct immunofluorescence study detected immunoglobulin M and C3 deposits in the papillary dermis. The patient responded well to oral aspirin and a prostaglandin analogue and was well controlled with a compression bandage. Vasculitic lesions in the underlying subcutis may have been overlooked in cases in which livedoid vasculopathy has been considered as a nonvasculitic disorder because our case demonstrates that livedoid vasculopathy can be accompanied by subcutaneous vasculitis.

摘要

萎缩性血管病已被公认为一种非血管炎性疾病,但在胶原病和结节性多动脉炎病例中,其皮下组织可出现真正的血管炎。我们报告一例42岁乙肝病毒携带者,患有萎缩性血管病并伴有皮下坏死性静脉炎。该患者还有15年强直性脊柱炎病史,目前病情缓解。皮肤病变表现为下肢浅表溃疡、紫癜、白色萎缩和网状红斑,皮肤活检显示真皮浅层血管周围有少量淋巴细胞浸润,管腔内有明显的纤维蛋白血栓和纤维蛋白沉积,符合萎缩性血管病的特征。然而,在皮下组织中发现了坏死性静脉炎,其特征为血管壁及其周围有大量淋巴细胞浸润,血管壁有明显的纤维蛋白样坏死。直接免疫荧光研究在乳头真皮中检测到免疫球蛋白M和C3沉积。患者对口服阿司匹林和前列腺素类似物反应良好,使用压迫绷带后病情得到良好控制。在将萎缩性血管病视为非血管炎性疾病的病例中,皮下的血管炎性病变可能被忽视了,因为我们的病例表明萎缩性血管病可伴有皮下血管炎。

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