Prado Renata, Brice Sylvia L, Fukuda Shunpei, Hashimoto Takashi, Fujita Mayumi
Department of Dermatology, University of Colorado Denver, Aurora, 80045, USA.
Arch Dermatol. 2011 Jan;147(1):67-71. doi: 10.1001/archdermatol.2010.362.
Pemphigus herpetiformis (PH) is a rare clinical entity that combines the clinical features of dermatitis herpetiformis and the immunopathologic features of pemphigus. The target antigen is usually desmoglein 1, with exceptional cases manifesting autoantibodies against desmoglein 3. More recently, it has been found that many patients with PH also demonstrate autoantibodies against desmocollin. The association of PH with a malignant neoplasm is rare.
We describe a patient with PH and a lung neoplasm. Immunologic studies demonstrated IgG antibodies to desmoglein 3 and to an unknown 178-kDa protein but no antibodies to desmocollin.
The association of PH with a thoracic malignant neoplasm has been reported in only 4 previous cases, and the neoplasm could be responsible for the unusual immunologic profile in the patient described herein. To our knowledge, this is the first report of PH with an associated neoplasm in which only anti-desmoglein 3 antibody was detected.
疱疹样天疱疮（PH）是一种罕见的临床疾病，它兼具疱疹样皮炎的临床特征和天疱疮的免疫病理特征。其靶抗原通常为桥粒芯糖蛋白1，少数病例表现为针对桥粒芯糖蛋白3的自身抗体。最近发现，许多PH患者还表现出针对桥粒胶蛋白的自身抗体。PH与恶性肿瘤的关联罕见。
我们描述了1例患有PH和肺部肿瘤的患者。免疫学研究显示存在针对桥粒芯糖蛋白3和一种未知的178 kDa蛋白的IgG抗体，但不存在针对桥粒胶蛋白的抗体。
此前仅报道过4例PH与胸部恶性肿瘤相关的病例，肿瘤可能是本文所述患者出现异常免疫谱的原因。据我们所知，这是首次报道仅检测到抗桥粒芯糖蛋白3抗体的与肿瘤相关的PH病例。
