Vezzoli Pamela, Parietti Michele, Carugno Andrea, Di Mercurio Marco, Benaglia Chiara, Zussino Martina, Cavalli Riccardo, Sena Paolo, Berti Emilio
Dermatology Unit, ASST Papa Giovanni XXIII Hospital, 24127 Bergamo, Italy.
Dermatology Unit, Department of Medical Sciences, University of Turin, 10124 Turin, Italy.
J Clin Med. 2022 Nov 21;11(22):6858. doi: 10.3390/jcm11226858.
pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age.
we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3.
the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.
寻常型天疱疮是一种罕见的自身免疫性水疱病,累及皮肤和黏膜,在儿童期很少发生。
我们报告一例来自布基纳法索的9岁儿童天疱疮病例,最初表现为口腔内对称分布的糜烂性病变。几个月后,我们还观察到背部色素沉着过度的病变。皮肤样本的组织病理学检查显示表皮内棘层松解,而直接免疫荧光显示表皮中有补体(C3)和免疫球蛋白G(IgG)沉积;酶联免疫吸附测定(ELISA)试验突出显示存在针对桥粒芯糖蛋白3的循环自身抗体。
COVID-19疫情的出现给该患者的随访带来了困难。然而,在接受全身类固醇和硫唑嘌呤联合治疗约一年后,患者达到临床缓解。