Department of Dermatology, Medical Military Academy, Saint Petersburg, Russia.
Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; and.
Am J Dermatopathol. 2020 Jun;42(6):452-454. doi: 10.1097/DAD.0000000000001567.
Intralymphatic histiocytosis is a rare reactive skin condition characterized by a nonspecific clinical presentation and, microscopically, by the collections of mononuclear histiocytes within the lumina of dilated lymphatic vessels. We report a rare case of intralymphatic histiocytosis associated with rosacea and prominent periocular edema (Morbihan disease). The patient is a 56-year-old woman with a 12-year history of rosacea who suddenly developed edema of the right upper eyelid that persisted 6 months before she sought medical advice. Because of an unclear clinical diagnosis, surgical excision of the edematous upper eyelid was performed. The histologic slides showed interstitial edema of the dermis with dilated vascular channels and small epithelioid cell granulomas around hair follicles. In addition, there were aggregates of cells inside numerous lymphatic vessels that were immunohistochemically positive for CD45, CD4, CD68, CD163, CD64, CD14, CD11c, and lysozyme and negative for CD3, CD20, CD30, CD56, S100, CD1a, and langerin.
淋巴管内组织细胞增生症是一种罕见的反应性皮肤疾病，其特征为非特异性临床表现，显微镜下可见扩张淋巴管腔内的单核组织细胞聚集。我们报告了一例罕见的与酒渣鼻和明显的眶周水肿（莫比汉病）相关的淋巴管内组织细胞增生症。患者为 56 岁女性，有 12 年酒渣鼻病史，其右侧上眼睑突然出现水肿，在寻求医疗建议前已持续 6 个月。由于临床诊断不明确，对水肿的上眼睑进行了手术切除。组织学切片显示真皮间质水肿，血管通道扩张，毛囊周围有小上皮样细胞肉芽肿。此外，在许多淋巴管内有细胞聚集，免疫组织化学显示这些细胞对 CD45、CD4、CD68、CD163、CD64、CD14、CD11c 和溶菌酶呈阳性，而对 CD3、CD20、CD30、CD56、S100、CD1a 和 langerin 呈阴性。
