Kordeva S, Broshtilova V, Batashki I, Tchernev G
1Onkoderma - Clinic for Dermatology, Venereology and Dermatologic Surgery, Sofia, Bulgaria.
2Department of Dermatology and Venereology, Military Medical Academy, Sofia, Bulgaria.
Georgian Med News. 2023 Jul-Aug(340-341):227-231.
Atrophoderma of Pasini and Pierini is a rare, considered benign, skin disease characterized by single or multiple asymptomatic atrophic plaques. Lesions can occur everywhere on the body with the trunk being the most often reported affected site. It appears in the second or third decade of life and affects mostly the female population, with male to female ratio of 1:6, commonly of white European descent. Different risk factors were described in the literature - genetic predisposition, infections with Epstein-Barr virus, varicella zoster and Borrelia burgdorferi, vaccinations, local trauma and more. Since the pandemic with COVID-19, skin manifestations after the viral infection with COVID-19 were reported. After a thorough search of the existing medical literature, we believe, we present the first case of a rapid progression of Atrophoderma of Pasini and Pierini after COVID-19 infection. Due to its similarity to morphea in some aspects, the condition is often misdiagnosed, and the proper treatment is often delayed. Sometimes the dilemma "Is it atrophoderma Pasini-Pierini or is it in fact morphea?" stays, but the exact histopathological verification and the "diagnostic clues" which can be used during the examination stage, are usually enough to diagnose the condition. We present a 63-year-old female with a rapid progression of atrophoderma of Pasini and Pierini after a COVID-19 infection. The lesion that she presented with was single, asymptomatic, with central hypopigmentation and slight atrophy, with a smooth, shiny surface and ivory color, and peripheral hyperpigmentation, measured 18x5cm, without the presence of perilesional erythema. The patient was initially diagnosed clinically with localized scleroderma (morphea) and treated with hydroxychloroquine 200 mg once daily for a 5-year period without improvement. Years later two biopsies from different lesional sites were taken, resulting in absence of sclerosis and dermal atrophy, but - reduction in the thickness of the dermis with fragmentation and hyalinization of collagen fibers forming a parallel orientation, dilated vascular vessels of small caliber and reduced number of skin appendages, confirming the diagnosis of atrophoderma Pasini-Pierini. The patient's therapy was switched to methotrexate with good therapeutic response. Often, the two conditions - morphea and atrophoderma of Pasini and Pierini can be mistaken due to its clinical similarity and sometimes coexistence. Therefore, we will shortly review the existing literature with key points on the similarities and differences.
帕西尼和皮耶里尼皮肤萎缩症是一种罕见的、被认为是良性的皮肤病,其特征为单个或多个无症状性萎缩斑块。皮损可发生于身体的任何部位,其中躯干是最常报道的受累部位。它出现在人生的第二个或第三个十年,主要影响女性人群,男女比例为1:6,常见于欧洲白人后裔。文献中描述了不同的危险因素——遗传易感性、感染爱泼斯坦-巴尔病毒、水痘带状疱疹病毒和伯氏疏螺旋体、接种疫苗、局部创伤等等。自新冠疫情以来,有关于感染新冠病毒后的皮肤表现的报道。在对现有医学文献进行全面检索后,我们认为,我们呈现了首例新冠病毒感染后帕西尼和皮耶里尼皮肤萎缩症快速进展的病例。由于其在某些方面与硬斑病相似,这种病症常被误诊,恰当的治疗也常常被延误。有时会存在“这是帕西尼-皮耶里尼皮肤萎缩症还是实际上是硬斑病?”的两难情况,但确切的组织病理学验证以及在检查阶段可使用的“诊断线索”通常足以诊断该病症。我们报告一名63岁女性,在感染新冠病毒后出现帕西尼和皮耶里尼皮肤萎缩症快速进展。她出现的皮损为单个、无症状,中央色素减退和轻微萎缩,表面光滑、有光泽,呈象牙色,周边色素沉着,大小为18×5厘米,无皮损周围红斑。患者最初临床诊断为局限性硬皮病(硬斑病),并接受羟氯喹200毫克每日一次治疗,为期5年但无改善。数年后,从不同皮损部位进行了两次活检,结果显示无硬化和真皮萎缩,但——真皮厚度变薄,胶原纤维断裂并玻璃样变,形成平行排列,小口径血管扩张,皮肤附属器数量减少,确诊为帕西尼-皮耶里尼皮肤萎缩症。患者的治疗改为甲氨蝶呤,治疗反应良好。通常,硬斑病和帕西尼和皮耶里尼皮肤萎缩症这两种病症由于临床相似性且有时并存而可能被混淆。因此,我们将简要回顾现有文献中关于它们异同点的要点。