Kindle Scott A, Hart Philip A, Chari Suresh T, Pittelkow Mark R, Smyrk Thomas C, Lehman Julia S
Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.
Am J Clin Dermatol. 2015 Apr;16(2):125-30. doi: 10.1007/s40257-014-0109-1.
Autoimmune pancreatitis is the prototypical manifestation of immunoglobulin G4-related disease, a fibroinflammatory syndrome that can affect virtually any organ. Rarely, skin involvement has been reported in immunoglobulin G4-related disease. Isolated case reports have described other distinct associated dermatoses.
Our objective was to determine the frequency and nature of dermatologic manifestations of immunoglobulin G4-related disease in patients with autoimmune pancreatitis.
Retrospective analysis of dermatologic conditions of patients with autoimmune pancreatitis.
Among the 118 identified patients, 31 (26.3%) had a dermatologic diagnosis [mean (standard deviation; SD) follow-up 5.9 (4.6) years]. Two patients (1.7%) had necrobiotic xanthogranuloma; three (2.5%) had another xanthomatous or xanthogranulomatous process. One patient (0.8%) had pemphigus erythematosus. No patient had immunoglobulin G4-related skin disease.
Skin involvement in immunoglobulin G4-related disease appears to be rare. A disproportionately high number of patients had xanthomatous or xanthogranulomatous processes, including necrobiotic xanthogranuloma. It remains unclear whether the association between immunoglobulin G4-related disease and necrobiotic xanthogranuloma or other xantho(granulo)matous processes represents shared pathophysiology, a mutual underlying driver, or coincidence, though the results of this study cast doubt on the latter. Although pemphigus was not reported in immunoglobulin G4-related disease previously, the prominent role of the immunoglobulin G4 subclass in each condition makes this association intriguing.
自身免疫性胰腺炎是免疫球蛋白G4相关疾病的典型表现，这是一种几乎可累及任何器官的纤维炎症综合征。免疫球蛋白G4相关疾病很少累及皮肤，已有个别病例报告描述了其他不同的相关皮肤病。
我们的目的是确定自身免疫性胰腺炎患者免疫球蛋白G4相关疾病的皮肤表现的频率和性质。
对自身免疫性胰腺炎患者的皮肤病情况进行回顾性分析。
在118例确诊患者中，31例（26.3%）有皮肤病诊断[平均（标准差；SD）随访5.9（4.6）年]。2例患者（1.7%）患有渐进性坏死性黄色肉芽肿；3例（2.5%）有其他黄色瘤或黄色肉芽肿性病变。1例患者（0.8%）患有红斑性天疱疮。没有患者患有免疫球蛋白G4相关皮肤病。
免疫球蛋白G4相关疾病累及皮肤似乎很少见。有相当高比例的患者患有黄色瘤或黄色肉芽肿性病变，包括渐进性坏死性黄色肉芽肿。免疫球蛋白G4相关疾病与渐进性坏死性黄色肉芽肿或其他黄色瘤（肉芽肿）性病变之间的关联究竟是代表共同的病理生理学、共同的潜在驱动因素还是巧合，目前尚不清楚，尽管本研究结果对后者提出了质疑。虽然之前免疫球蛋白G4相关疾病中未报告天疱疮，但免疫球蛋白G4亚类在每种疾病中的显著作用使得这种关联很有趣。
