Coexistence of antilaminin-332-type mucous membrane pemphigoid, lamina lucida-type linear IgA bullous dermatosis and Sjogren syndrome.
Coexistence of antilaminin-332-type mucous membrane pemphigoid, lamina lucida-type linear IgA bullous dermatosis and Sjogren syndrome.
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抗层粘连蛋白332型粘膜类天疱疮、透明板型线性IgA大疱性皮肤病和干燥综合征并存。
DOI：
10.1111/ced.12030
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Shimizu T
中科院分区：
文献类型：
--
作者：
Takegami Y;Makino T;Matsui K;Ueda C;Fukuda S;Hashimoto T;Shimizu T
Sjögren综合征（SS）通常与其他自身免疫性疾病相关，如系统性红斑狼疮。1近年来，偶有不同自身免疫性水疱病并存的病例报道；然而，SS和自身免疫性表皮下水疱病共存是罕见的。我们报告一例粘膜类天疱疮（MMP）和线状IgA大疱性皮肤病（LABD）并发SS。一名71岁的日本女性，表现为红斑，瘙痒性水疱和糜烂。她没有相关的个人或家族病史，也没有服用任何药物。体格检查中，患者的眼睑、嘴唇（图1a）和口腔粘膜可见糜烂和结痂，但未见瘢痕形成。30 - 50mm大小的环状红斑斑块广泛分布于躯干和四肢（图1b），红斑周围可见多个紧张性水泡（图1c）。在对患者背部皮肤活检标本进行组织学检查时，可见表皮下分离，真皮上部有嗜酸性粒细胞和中性粒细胞浸润（图1d）。(a) (b) (c) (d) (e) (f) (g) (h) 50μm 50μm 50μm 50μm50μm
Sjögren syndrome (SS) is often associated with other autoimmune disorders, such as systemic lupus erythematosus. 1 Recently, some cases with coexistence of different autoimmune blistering diseases have been occasionally reported; however, the coexistence of SS and autoimmune subepidermal blistering disease is rare. 2, 3 We report a case of mucous membrane pemphigoid (MMP) and linear IgA bullous dermatosis (LABD) coexisting with SS. A 71-year-old Japanese woman presented with erythema, pruritic tense blisters and erosions. She had no relevant personal or family medical history, and was not taking any medication. On physical examination, erosions and crusts were seen on the patient’s eyelids, lips (Fig. 1a) and oral mucosae, but without scarring. Annular erythematous patches 30–50 mm in size were distributed widely over the trunk and limbs (Fig. 1b) and multiple tense blisters were seen on the periphery of the erythema (Fig. 1c). On histological examination of a skin biopsy specimen obtained from the patient’s back, subepidermal separation was seen, with an infiltration of eosinophils and neutrophils in the upper dermis (Fig. 1d).(a)(b)(c)(d)(e)(f)(g)(h)50 μm 50 μm 50 μm 50 μm50 μm