Vornicescu Corina, Șenilă Simona Corina, Cosgarea Rodica, Candrea Elisabeta, Pop Alexandra Dana, Ungureanu Loredana
Department of Dermatology, 'Iuliu Hațieganu' University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania.
Department of Physiology, 'Iuliu Hațieganu' University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania.
Exp Ther Med. 2019 Feb;17(2):1132-1138. doi: 10.3892/etm.2018.7057. Epub 2018 Dec 5.
Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patients, with long mean duration of symptoms, the correct diagnosis of PN is delayed because the disease is not recognized. We present a case and summarize the reported characteristics of PN. The search in MEDLINE database, after selection, resulted in 36 articles presenting 47 cases of PN. Between published cases a female predominance was noted (female to male ratio of 1.8:1), almost half of the reported patients were non-Caucasian, and the mean age at presentation was 66.2 years. The mean duration until the diagnosis was almost 2 years. Sixteen patients also had other autoimmune diseases. Twenty-two patients developed vesicles/bullae/urticarial plaques before or after the diagnosis. Peripheral eosinophilia and high levels of serum total IgE were reported in 10.6 and 27.2% of patients, respectively. ELISA for either BP180, BP230 or both were positive in all tested cases. DIF and IIF microscopy were positive overall in 100 and 92.3% of cases, respectively. Corticosteroids, either topical or systemic, were the most efficient therapeutic option, although many others were used. PN remains a diagnostic and therapeutic challenge in elderly patients with unexplained refractory chronic pruritus associated with papulo-nodular lesions.
结节性类天疱疮（PN）是大疱性类天疱疮的一种罕见临床变体，其特征为存在结节性痒疹样皮损和类天疱疮水疱。通过直接免疫荧光（DIF）/间接免疫荧光（IIF）和免疫血清学检测可确诊。对于一些症状平均持续时间较长的患者，由于该疾病未被认识，PN的正确诊断会延迟。我们报告一例病例并总结已报道的PN特征。在MEDLINE数据库中筛选后，得到36篇文章，共报道47例PN。在已发表的病例中，女性占优势（女性与男性比例为1.8:1），几乎一半的报道患者为非白种人，就诊时的平均年龄为66.2岁。诊断前的平均病程近2年。16例患者还患有其他自身免疫性疾病。22例患者在诊断之前或之后出现水疱/大疱/荨麻疹斑块。分别有10.6%和27.2%的患者报告有外周血嗜酸性粒细胞增多和血清总IgE水平升高。在所有检测病例中，针对BP180、BP230或两者的酶联免疫吸附测定（ELISA）均为阳性。DIF和IIF显微镜检查总体阳性率分别为100%和92.3%。皮质类固醇，无论是局部用药还是全身用药，都是最有效的治疗选择，尽管也使用了许多其他药物。对于患有无法解释的难治性慢性瘙痒并伴有丘疹结节性皮损的老年患者，PN仍然是一个诊断和治疗难题。
