Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus
Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus
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DOI：
10.1111/j.1365-2230.2012.04468.x
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发表时间：
2013-01-01
影响因子：
4.1
通讯作者：
Aoki, V.
中科院分区：
文献类型：
--
作者：
Oliveira Junior, J. V.;Maruta, C. W.;Aoki, V.
背景资料。寻常型天疱疮(PV)和叶型天疱疮(PF)是一种自身免疫性囊泡性疾病，具有针对桥粒芯糖蛋白(DSG)1和3的自身抗体，可导致表皮内棘层松解。瞄准。目的探讨脐带受累的PF或PV患者的临床和免疫学特征。方法：研究方法。总共10例患者(女性7例，男性3例；年龄2470岁，病程316年)被诊断为PV(n=5)或粘膜皮肤PF(n=5)，根据其临床特征、组织病理学和免疫学结果[直接和间接免疫荧光(DIF和IIF)以及重组DSG1和DSG3的ELISA法]进行评估。结果。红斑、糜烂、结痂、植物皮损是脐区的主要临床特征。8例脐区DIF阳性，8例阳性，另2例仅阳性。免疫球蛋白标记的间接免疫荧光显示10例患者均呈典型的天疱疮模式，滴度从1：160到1：2560不等。用重组DSG1进行酶联免疫吸附试验，PF得24266分，PV得0270分。重组Dsg3在5例PV患者中均为阳性(ELISA2298)，而在所有PF血清中均为阴性。结论。10例以脐带为首发症状的天疱疮患者均具有PF或PV的临床和免疫病理特征。这种尚未完全阐明的特殊表现在文献中很少被报道。这一独特表现的可能解释可能是存在新的表位，或者与位于脐带区的胚胎或疤痕组织有关。
Background. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune vesicobullous disorders with IgG autoantibodies directed against desmoglein (Dsg)1 and 3, which lead to intraepidermal acantholysis. Aim. To characterize the clinical and immunological profile of patients with PF or PV with umbilical involvement. Methods. In total, 10 patients (7 women, 3 men; age range 2470 years, disease duration 316 years) diagnosed with either PV (n = 5) or mucocutaneous PF (n = 5) were assessed according to their clinical features, histopathology and immunological findings [direct and indirect immunofluorescence (DIF and IIF) and ELISA with recombinant Dsg1 and Dsg3]. Results. Erythema, erosions, crusts and vegetating skin lesions were the main clinical features of the umbilical region. DIF of the umbilical region gave positive results for intercellular epidermal IgG and C3 deposits in eight patients and for IgG alone in the other two. Indirect immunofluorescence with IgG conjugate showing the typical pemphigus pattern was positive in all 10 patients, with titres varying from 1 : 160 to 1 : 2560. ELISA with recombinant Dsg1 gave scores of 24266 in PF and 0270 in PV. Reactivity to recombinant Dsg3 was positive in all five patients with PV (ELISA 2298) and was negative in all PF sera. Conclusions. All 10 patients with pemphigus with umbilical presentation had the clinical and immunopathological features of either PF or PV. This peculiar presentation, not yet completely elucidated, has rarely been reported in the literature. A possible explanation for this unique presentation may be the presence of either novel epitopes or an association with embryonic or scar tissue located in the umbilical-cord region.