IgA pemphigus with non-pustular erythematous lesions and IgA antibodies to desmocollins 1 and 2
IgA pemphigus with non-pustular erythematous lesions and IgA antibodies to desmocollins 1 and 2
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DOI：
10.1684/ejd.2013.2046
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发表时间：
2013-05-01
影响因子：
2.5
通讯作者：
Hashimoto, Takashi
中科院分区：
文献类型：
--
作者：
Arai, Rie;Okuda, Hiroto;Hashimoto, Takashi
IgA天疱疮是天疱疮的一种罕见变种。IGA天疱疮分为表皮内中性粒细胞IgA皮肤病(IEN型)和角膜下脓疱性皮肤病(Dsc1)，前者的靶抗原仍是一个谜。我们报告一位56岁的日本男性，患有不典型红斑的IgA天疱疮。红斑出现一个月后，病人去看他的私人医生，初步诊断为多形性红斑。患者在来本院就诊前曾间歇性服用小剂量强的松龙治疗一年，但未见疗效。临床检查显示躯干和四肢有不规则形状和部分水肿性红斑，无粘膜受累。治疗过程中均未见大疱疹和脓疱疹。直接免疫荧光显示表皮上2/3的角质形成细胞表面有IgA沉积。猴食道切片间接免疫荧光检测到抗细胞表面抗体IgA和Ig G。我们的新的酶联免疫吸附试验使用人DSC1-3的真核重组蛋白来检测Dsc1和Dsc2的IgA抗体。虽然没有观察到明显的水泡，但诊断为IgA天疱疮。强的松龙每天需要30 mg来控制红斑性皮损。虽然这种独特的皮肤损害的发病机制尚不清楚，但考虑到IgA天疱疮有前驱期的可能性，以及早期使用小剂量强的松龙抑制脓疱或大疱病的发展的可能性。
IgA pemphigus is a rare variant of pemphigus. IgA pemphigus is subdivided into intraepidermal neutrophilic IgA dermatosis-type (IEN-type), whose target antigen is still an enigma, and subcorneal pustular dermatosis-type, whose target antigen is desmocollin 1 (Dsc1). We report a 56-year-old Japanese male with IgA pemphigus showing atypical erythema. One month after erythema developed, the patient visited his private physician, and was tentatively diagnosed as having erythema multiforme. The patient had been intermittently treated with a low dose of oral prednisolone for a year without benefit before visiting our hospital. Clinical examination revealed irregularly-shaped and partially edematous erythema over the trunk and extremities without mucosal involvement. Neither bullae nor pustules were seen during the course. Direct immunofluorescence showed IgA deposition on cell surfaces of keratinocytes in the upper two thirds of the epidermis. Indirect immunofluorescence of monkey esophagus sections revealed IgA and IgG anti-cell surface antibodies. Our new enzyme-linked immunosorbent assays using eukaryotic recombinant proteins of human Dsc 1-3 detected IgA antibodies to Dsc1 and Dsc2. Although no apparent bullae were observed, the diagnosis of IgA pemphigus was made. Prednisolone 30 mg daily was required to control erythematous lesions. Although the pathomechanism for the unique skin lesion is unknown, the possibility that IgA pemphigus has a prodromal phase and that early administration of low dose prednisolone suppressed the development of pustules or bullae were considered.