Mimouni D, Ng P P, Rencic A, Nikolskaia O V, Bernstein B D, Nousari H C
Department of Dermatology, Division of Immunodermatology, Johns Hopkins University School of Medicine, Suite 771, Ross Research Building, 720 Rutland Avenue, Baltimore, MD 21205, USA.
Br J Dermatol. 2003 Apr;148(4):789-94. doi: 10.1046/j.1365-2133.2003.05176.x.
The term 'atrophie blanche' is used both as a descriptive term denoting ivory-white stellate scars on the lower limbs as well as a diagnostic label synonymous with livedoid vasculitis, an ill-defined entity. Medium-sized vasculitides, such as polyarteritis nodosa (PAN), occasionally present with ulceration resulting in ivory-white stellate scarring on the lower limbs and may potentially be misdiagnosed as livedoid vasculitis.
To assess the occurrence, clinical and immunopathological features of medium-sized vasculitis in patients presenting with atrophie blanche without clinical and/or compression duplex ultrasonographic evidence of venous insufficiency.
We retrospectively evaluated patients presenting with atrophie blanche at the Department of Dermatology of Johns Hopkins Medical Institutions, from April 1996 until April 2002, following the diagnostic guidelines for leg ulcers of the Division of Immunodermatology. Deep and multiple skin biopsies were performed for histology. Investigations for underlying vasculitis, thrombophilia, nerve conduction studies and compression duplex ultrasonography of the lower extremities were performed in all patients.
Of 29 consecutive patients presenting with atrophie blanche, six had underlying medium-sized vasculitis consistent with PAN, three of whom had previously been diagnosed to have segmental hyalinizing vasculitis/vasculopathy (livedoid vasculitis/vasculopathy) on superficial biopsies. All six patients with cutaneous PAN were women with a median age of 36.5 years (range 34-46) and with a median duration of the disease prior to diagnosis of 18 years (range 3-30). Of the six cutaneous PAN patients, four had neurological involvement evidenced by clinical symptoms and nerve conduction studies. No evidence of any other extracutaneous involvement was found. Erythrocyte sedimentation rate and tests for vasculitis and thrombophilic were normal in all six patients. None had evidence of venous insufficiency. Immunosuppressive therapy was effective in controlling PAN-associated cutaneous and neurological disease. Of the remaining 23 patients, two had antiphospholipid syndrome and one had homocystineaemia; all three also had evidence of venous insufficiency. One patient had multiple myeloma-associated type I cryoglobulinaemia and 19 patients had venous insufficiency alone. None of the non-PAN patients had abnormalities in the nerve conduction studies.
In patients presenting with atrophie blanche without evidence of venous insufficiency and thrombophilia, PAN should be excluded, particularly in the presence of mononeuritis multiplex. Repeated and deep biopsies are often necessary to reveal the accurate underlying pathology of necrotizing medium-sized vasculitis in the reticular dermis and the subcutis, especially in the setting of atrophie blanche lesions. Immunosuppressive therapy was effective in controlling the PAN-associated clinical manifestations.
“白色萎缩”一词既作为描述性术语表示下肢象牙白色星状瘢痕，也作为与类脂质渐进性坏死性血管炎同义的诊断标签，这是一种定义不明确的疾病。中等大小血管炎，如结节性多动脉炎（PAN），偶尔会出现溃疡，导致下肢象牙白色星状瘢痕，可能会被误诊为类脂质渐进性坏死性血管炎。
评估无静脉功能不全临床和/或压迫性双功超声证据的白色萎缩患者中中等大小血管炎的发生率、临床和免疫病理特征。
我们回顾性评估了1996年4月至2002年4月在约翰霍普金斯医疗机构皮肤科就诊的白色萎缩患者，遵循免疫皮肤病学分部的腿部溃疡诊断指南。进行了深部和多处皮肤活检以进行组织学检查。对所有患者进行了潜在血管炎、血栓形成倾向、神经传导研究和下肢压迫性双功超声检查。
在连续29例白色萎缩患者中，6例有与PAN一致的潜在中等大小血管炎，其中3例先前在浅表活检中被诊断为节段性透明变性血管炎/血管病（类脂质渐进性坏死性血管炎/血管病）。所有6例皮肤PAN患者均为女性，中位年龄36.5岁（范围34 - 46岁），诊断前疾病中位持续时间为18年（范围3 - 30年）。在6例皮肤PAN患者中，4例有临床症状和神经传导研究证明的神经受累。未发现任何其他皮肤外受累的证据。所有6例患者的红细胞沉降率以及血管炎和血栓形成倾向检测均正常。均无静脉功能不全的证据。免疫抑制治疗有效控制了与PAN相关的皮肤和神经疾病。在其余23例患者中，2例有抗磷脂综合征，1例有高同型半胱氨酸血症；所有3例也有静脉功能不全的证据。1例患者有多发性骨髓瘤相关的I型冷球蛋白血症，19例患者仅有静脉功能不全。非PAN患者均无神经传导研究异常。
在无静脉功能不全和血栓形成倾向证据的白色萎缩患者中，应排除PAN，特别是在有多发性单神经炎的情况下。重复和深部活检通常对于揭示网状真皮和皮下组织中坏死性中等大小血管炎的准确潜在病理是必要的，特别是在白色萎缩病变的情况下。免疫抑制治疗有效控制了与PAN相关的临床表现。
