Department of Dermatology, The University of Texas Health Science Center at Houston, Houston, Texas, USA.
Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
J Cutan Pathol. 2022 Jul;49(7):658-662. doi: 10.1111/cup.14221. Epub 2022 Mar 21.
Epidermodysplasia verruciformis (EDV) is a rare genodermatosis that predisposes affected individuals to persistent infection with certain types of human papillomavirus (HPV), particularly those that belong to the genus beta-HPV, including HPV-5 and HPV-8, which carry high oncogenic potential. There are three main HPV-related viral cytopathic changes in cutaneous verrucae in terms of intracytoplasmic inclusion bodies (ICBs), namely, granular, filamentous, and homogeneous type ICBs. To date, only HPV-4, HPV-60, and HPV-65 have been found in association with homogeneous ICBs. We report a unique case of HPV-49-associated EDV in a 41-year-old woman with common variable immunodeficiency, mycosis fungoides, and multiple cutaneous malignancies, including squamous cell carcinoma and Merkel cell carcinoma who presented with multiple pink papules and hyperpigmented macules on the left upper extremity. One of the skin lesions histopathologically revealed keratinocytic nuclear enlargement with abundant blue-gray cytoplasm, accompanied by hypergranulosis, characteristic of EDV, along with peculiar bright eosinophilic and homogeneous ICBs. To the best of our knowledge, this is the first reported case of EDV with detection of HPV-49 by genotyping, which features eosinophilic homogeneous ICBs, like those seen in the setting of HPV-4, HPV-60, or HPV-65 infection.
疣状表皮发育不良(EDV)是一种罕见的遗传性皮肤病,使受影响的个体易感染某些类型的人乳头瘤病毒(HPV),特别是那些属于β-HPV 属的 HPV,包括 HPV-5 和 HPV-8,它们具有高致癌潜能。在皮肤疣的细胞内包涵体(ICB)方面,有三种主要的 HPV 相关病毒细胞病变,即颗粒状、丝状和均质型 ICB。迄今为止,只有 HPV-4、HPV-60 和 HPV-65 与均质 ICB 有关。我们报告了一例独特的 HPV-49 相关 EDV 病例,患者为 41 岁女性,患有普通可变免疫缺陷、蕈样真菌病和多种皮肤恶性肿瘤,包括鳞状细胞癌和 Merkel 细胞癌,左上肢有多个粉红色丘疹和色素沉着斑。其中一个皮肤病变的组织病理学显示角质形成细胞核增大,伴有丰富的蓝灰色细胞质,伴有过度角化,具有 EDV 的特征,以及独特的明亮嗜酸性和均质 ICB。据我们所知,这是首例通过基因分型检测到 HPV-49 的 EDV 病例,其特征为嗜酸性均质 ICB,类似于 HPV-4、HPV-60 或 HPV-65 感染时所见。