Department of Dermatology, Immunodermatology and Venereology, Medical University of Warsaw, Warsaw, Poland.
Front Immunol. 2022 Apr 12;13:885023. doi: 10.3389/fimmu.2022.885023. eCollection 2022.
Pemphigoid nodularis is a rare form of pemphigoid that joins the clinical picture of prurigo nodularis and the immunological features of bullous pemphigoid, which is therapeutically challenging. Here, we analyze five female patients with a long-lasting course of nodular pemphigoid in terms of clinical and immunological characteristics and therapy. All the patients fulfilled clinical and immunological criteria of nodular pemphigoid. We applied numerous techniques allowing the proper diagnosis: direct and indirect immunofluorescence, salt split skin, ELISA, BIOCHIP, and fluorescence overlay antigen mapping using laser scanning confocal microscopy. Our study showed that 4 of 5 patients fulfilled the clinical and immunological criteria of nodular bullous pemphigoid. Two out of 4 patients presented exclusively nodular lesions; in the other two patients, blisters and erythematous lesions preceded prurigo-like lesions by a few years. The remaining patient had clinical and immunological criteria of nodular mucous membrane pemphigoid, presenting oral erosions, scarring conjunctivitis, and numerous disseminated nodules on the skin. All the patients were treated with multiple medicines; however, it was observed that the use of clobetasol propionate on the entire body plus antidepressants best controlled the disease. Pemphigoid nodularis mainly occurs in elderly women. In cases with coexisting psychological problems, antidepressants should be considered as an important complementary therapy to the basic one with clobetasol propionate.
结节性类天疱疮是一种罕见的类天疱疮，兼具慢性单纯性痒疹的临床表现和大疱性类天疱疮的免疫特征，治疗极具挑战性。在此，我们分析了 5 例女性患者的结节性类天疱疮的临床和免疫特征及治疗情况。所有患者均符合结节性类天疱疮的临床和免疫标准。我们应用了多种技术进行诊断：直接和间接免疫荧光、盐裂皮肤、ELISA、BIOCHIP 和激光扫描共聚焦显微镜荧光重叠抗原定位。研究表明，5 例患者中有 4 例符合结节性大疱性类天疱疮的临床和免疫标准。4 例患者中有 2 例仅表现为结节性病变，另外 2 例患者在出现瘙痒样丘疹样皮损前几年先出现水疱和红斑性皮损。另一位患者符合结节性黏膜类天疱疮的临床和免疫标准，表现为口腔糜烂、瘢痕性结膜炎和皮肤多发播散性结节。所有患者均接受了多种药物治疗，但观察到全身使用丙酸氯倍他索加用抗抑郁药可更好地控制疾病。结节性类天疱疮主要发生在老年女性中。对于同时存在心理问题的患者，应考虑将抗抑郁药作为基本治疗（丙酸氯倍他索）的重要补充治疗。
