Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid.
Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid.
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红皮病性大疱性类天疱疮是大疱性类天疱疮的一种临床变异型。
DOI：
10.1111/j.1365-2133.1995.tb06935.x
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发表时间：
1995
期刊：
影响因子：
--
通讯作者：
Woods,SG
中科院分区：
文献类型：
--
作者：
Korman,NJ;Woods,SG
大疱性类天疱疮（BP）是一种自身免疫性皮肤起泡疾病。已经描述了几种变体olBP，但直到最近，这些变体与广义BP的关系尚不清楚。几项研究表明，结节性类天疱疮、增殖性类天疱疮、局限性BP和水疱性类天疱疮是BP的真正变体，因为这些患者的循环抗体识别与全身性BP患者相同的230 kDa BP抗原。红皮病型BP是一种非常罕见的变异型，其特征为红皮病沿着水疱形成。我们描述了第三个已知的患者，以发展红皮病BP和表征的抗原特异性循环抗体在我们的新报告的红皮病BP患者和其他两个先前报道的红皮病BP病例之一。两名红皮病BP患者均具有循环IgG抗体，其以与两名免疫病理学证实的全身性BP患者相同的模式结合至盐裂人皮肤的表皮侧。作为对照，来自四名无水疱的红皮病患者和来自健康正常志愿者的血清未能证明可检测的循环IgG自身抗体。免疫沉淀研究表明，红皮病BP患者循环IgG自身抗体的识别，在不同程度上，相同的230和180 kDa的BP抗原，从两名患者的血清免疫病理学证实广义BP。作为对照，来自四名无水疱的红皮病患者和来自健康正常志愿者的血清未能识别任何特异性多肽。这些观察结果表明，红皮病BP是BP的一个独特的临床变异。
Bullous pemphigoid (BP) is an autoimmune blistering disease of the skin. Several variants olBP have been described but until recently the relationship of these variants to generalized BP was unclear. Several studies have shown that pemphigoid nodularis, pemphigoid vegetans, localized BP and vesicular pemphigoid are true variants of BP as the circulating antibodies in these patients recognize the same 230kDa BP antigen as found in patients with generalized BP. Erythrodermic BP is a very unusual variant characterized by an erythroderma along with blister formation. We describe the third known patient to develop erythrodermic BP and characterize the antigenic specificity of the circulating antibodies in both our newly reported patient with erythrodermic BP and in one of the two other previously reported cases of erythrodermic BP. Both patients with erythrodermic BP had circulating IgG antibodies which bound to the epidermal side of salt‐split human skin in a pattern identical to two patients with immunopathologically proven generalized BP. Sera from four erythrodermic patients without blisters and from a healthy normal volunteer, as controls, failed to demonstrate detectable circulating IgG autoantibodies. Immunoprecipitation studies revealed that both patients with erythrodermic BP had circulating IgG autoantibodies which recognized, to varying degrees, the same 230 and 180kDa BP antigens as recognized by sera from two patients with immunopathologically proven generalized BP. Sera from four erythrodermic patients without blisters and from a healthy normal volunteer, as controls, failed to recognize any specific polypeptides. These observations demonstrate that erythrodermic BP is a distinct clinical variant of BP.