State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, Department of Oral Medicine, West China Hospital of Stomatology, Sichuan University, Chengdu, Sichuan, China.
Dermatovenereology Department, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, China.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2021 Aug;132(2):e62-e68. doi: 10.1016/j.oooo.2021.02.004. Epub 2021 Feb 11.
Pemphigus vegetans is a rare variant of pemphigus vulgaris. Here, we report a rare case of postparturition pemphigus vegetans in a young woman without any prior medical history. The patient presented with extensive oral mucosal erosion, conjunctivitis, and fingernail dystrophy. A histopathological biopsy taken from the tongue showed pseudoepitheliomatous hyperplasia of the epidermis with eosinophilic spongiosis, and an intraepidermal cleft and direct immunofluorescence showed intercellular immunoglobulin G and complement component C3 deposition. A final diagnosis of pemphigus vegetans was made on the basis of clinical and histopathological features and laboratory findings. Complete remission was achieved by a combination of methylprednisolone and thalidomide. This case report indicates that the occurrence of postparturition pemphigus vegetans is strongly associated with immune status related to hormone levels throughout pregnancy and postparturition. Furthermore, it suggests that a combination of methylprednisolone and thalidomide may be an effective option for pemphigus vegetans treatment.
落叶型天疱疮是寻常型天疱疮的一种罕见变异型。在此,我们报告一例年轻女性产后罹患落叶型天疱疮的罕见病例,该患者既往无任何病史。患者表现为广泛的口腔黏膜糜烂、结膜炎和指甲营养不良。取自舌部的组织病理学活检显示表皮呈假上皮瘤样增生,伴嗜酸性海绵形成,表皮内裂隙,直接免疫荧光显示细胞间免疫球蛋白 G 和补体成分 C3 沉积。基于临床、组织病理学特征和实验室检查结果,最终诊断为落叶型天疱疮。甲泼尼龙和沙利度胺联合治疗实现完全缓解。本病例报告表明,产后落叶型天疱疮的发生与与妊娠和产后激素水平相关的免疫状态密切相关。此外,这表明甲泼尼龙和沙利度胺联合治疗可能是落叶型天疱疮的有效治疗选择。