Viseux V, Schoenlaub P, Marciniak S, Staroz F, Plantin P
Service de Dermatologie, CHIC, Hôpital Laënnec, Quimper.
Ann Dermatol Venereol. 2003 Dec;130(12 Pt 1):1150-3.
Wells' syndrome is a dermatological disease with polymorphous lesions characterized histologically by an eosinophilic infiltrate of the dermis with edema and flame figures. Kikuchi's disease is a benign cause of lymphadenitis corresponding histologically to a necrotizing histiocyting adenitis without neutrophils. We describe the association of a Kikuchi's disease and cutaneous lesions similar to Wells'syndrome.
A 62-year-old man presented over 3 years several simultaneous episodes of left axillary lymphadenitis and of cutaneous lesions compatible with a diagnosis of Wells'syndrome. No precipitating event or disease described with the Wells'syndrome was established. Concerning the axillary nodes, a cancer, a lymphoproliferative syndrome, a systemic lupus and several infectious diseases were excluded. Standard histology and immunochemistry of a lymph node showed signs of Kikuchi's disease. Bilateral anterior uveitis was incidentally detected. The three conditions improved with oral corticosteroids (1 mg/kg/d).
The patient had Kikuchi's disease and a recurrent dermatosis for which the clinical and histological aspects, the evolution and the absence of arguments for another cause, suggest a Wells'syndrome. Cutaneous manifestations occur in 16 to 40 p. 100 of cases of Kikuchi's disease and often have characteristic histologic features not corresponding to Wells'syndrome. The association of the 2 diseases may be then incidental, despite 4 simultaneous episodes. Two viral agents, VIH and VZV have been associated with the two diseases but do not seem to be the cause in our patient. Concerning the anterior uveitis, a single case of uveitis has been described for each condition.
韦尔斯综合征是一种具有多形性皮损的皮肤病,组织学特征为真皮嗜酸性粒细胞浸润伴水肿和火焰状图形。菊池病是淋巴结炎的一种良性病因,组织学上对应于无中性粒细胞的坏死性组织细胞性淋巴结炎。我们描述了一例菊池病与类似韦尔斯综合征的皮肤损害相关的病例。
一名62岁男性在3年多的时间里多次同时出现左腋窝淋巴结炎和符合韦尔斯综合征诊断的皮肤损害。未发现与韦尔斯综合征相关的诱发事件或疾病。关于腋窝淋巴结,排除了癌症、淋巴增殖综合征、系统性红斑狼疮和几种传染病。淋巴结的标准组织学和免疫化学检查显示有菊池病的迹象。偶然发现双侧前葡萄膜炎。这三种情况经口服皮质类固醇(1mg/kg/d)后有所改善。
该患者患有菊池病和一种复发性皮肤病,其临床和组织学表现、病情演变以及缺乏其他病因的依据,提示为韦尔斯综合征。皮肤表现见于16%至40%的菊池病病例,且通常具有与韦尔斯综合征不符的特征性组织学特征。尽管有4次同时发作,但这两种疾病的关联可能是偶然的。两种病毒,即艾滋病毒和水痘带状疱疹病毒,已被认为与这两种疾病有关,但在我们的患者中似乎不是病因。关于前葡萄膜炎,每种疾病各有一例葡萄膜炎的描述。