Ishibashi Masafumi, Miyamoto Juria, Nagasaka Takeshi, Chen Ko-Ron
Department of Dermatology, Saiseikai Central Hospital, Tokyo, Japan.
Am J Dermatopathol. 2009 May;31(3):293-6. doi: 10.1097/DAD.0b013e31819560aa.
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.
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。