Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris
Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris
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DOI：
10.1046/j.1365-2133.2002.04969.x
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发表时间：
2002-10-01
影响因子：
10.3
通讯作者：
Camps, A
中科院分区：
文献类型：
--
作者：
Campo-Voegeli, A;Muñiz, F;Camps, A
结合桥粒芯糖蛋白（Dsg）1和Dsg 3的分布模式和抗Dsg自身抗体谱（Dsg补偿理论）可以很好地解释天疱疮的临床表型。据报道，新生儿皮肤具有与成人粘膜上皮相似的Dsg分布模式。我们描述了一个新生儿女孩与粘膜皮肤寻常天疱疮（PV）的母亲与粘膜占主导地位的PV。母亲有疼痛的口腔糜烂至少7个月。组织学检查和直接和间接免疫荧光研究证实了PV和新生儿PV的诊断，在母亲和女儿，分别。母亲有高滴度的抗Dsg3 IgG和低滴度的抗Dsg1 IgG，而新生儿只有高滴度的抗Dsg3 IgG，但没有检测到抗Dsg1 IgG。抗Dsg3 IgG，导致母亲的口腔显性表型，诱导广泛的口腔以及新生儿的皮肤病变。本病例为新生儿肺静脉Dsg代偿学说提供了临床依据。
The clinical phenotype of pemphigus is well explained by the combination of desmoglein (Dsg) 1 and Dsg3 distribution pattern and antiDsg autoantibody profile (Dsg compensation theory). It has been reported that neonatal skin has a similar Dsg distribution pattern to adult mucosal epithelia. We describe a newborn girl with mucocutaneous pemphigus vulgaris (PV) from a mother with mucosal dominant PV. The mother had had painful oral erosions for at least 7 months. Histopathological examination and direct and indirect immunofluorescence studies confirmed the diagnosis of PV and neonatal PV in the mother and daughter, respectively. The mother had a high titre of anti-Dsg3 IgG and a low titre of antiDsg1 IgG, while the neonate had only a high titre of anti-Dsg3 IgG, but no detectable antiDsg1 IgG. AntiDsg3 IgG, which caused the oral dominant phenotype in the mother, induced extensive oral as well as cutaneous lesions in the neonate. Our case provides clinical evidence for the Dsg compensation theory in neonatal PV.