Ramassamy Sivaranjini, Van Hoang Thi Ai, Chuang Jen-Yu, Wu Yu-Hung
Assistant Professor, Department of Dermatology and STD, Jawaharlal Institute of Post-graduate Medical Education and Research, Puducherry, India.
Attending Dermatologist, Department of Dermatology, Da Nang Hospital of Dermatology and Venereology, Da Nang, Vietnam.
Am J Dermatopathol. 2022 Feb 1;44(2):83-91. doi: 10.1097/DAD.0000000000001952.
Cheilitis granulomatosa (CG) is an idiopathic, rare, and chronic granulomatous disorder involving the lips. We characterized the pathological and immunohistopathological findings of these granulomas and their relationship with the lymphatic vessels. Pathologically confirmed cases of primary CG from 2001 to 2016 were collected. Cases of inflammatory cheilitis without the presence of granuloma were included in the control group. Demographic data, clinical presentation, response to therapy, and pathological differences were compared. Periodic acid-Schiff and acid-fast stains excluded patients having infections. CD68, CD163, and D2-40 stains demonstrated features of granuloma, macrophage polarization, and the relationship between granuloma and lymphatic vessels. Thirteen patients diagnosed with CG were enrolled. Thirteen people were enrolled in the control group. The granulomas were either mononuclear or sarcoidal. They were predominantly positive for CD68 but negative for CD163. Perilymphatic granulomas were found in all patients. Intralymphatic histiocytosis and lymphatic dilatation were more commonly observed in patients diagnosed with CG than those in controls (54% vs. 15%, P = 0.03 and 92% vs. 23%, P < 0.01). TH1 immune response due to CD68+ M1 macrophages results in CG. Perilymphatic aggregation of macrophages and intralymphatic histiocytosis were important pathological clues for diagnosis.
肉芽肿性唇炎（CG）是一种累及唇部的特发性、罕见的慢性肉芽肿性疾病。我们对这些肉芽肿的病理和免疫组化结果及其与淋巴管的关系进行了特征描述。收集了2001年至2016年经病理证实的原发性CG病例。对照组纳入无肉芽肿的炎性唇炎病例。比较了人口统计学数据、临床表现、治疗反应和病理差异。过碘酸希夫染色和抗酸染色排除了有感染的患者。CD68、CD163和D2-40染色显示了肉芽肿的特征、巨噬细胞极化以及肉芽肿与淋巴管的关系。纳入了13例诊断为CG的患者。对照组纳入了13人。肉芽肿为单核或结节状。它们主要CD68阳性但CD163阴性。所有患者均发现淋巴管周围肉芽肿。与对照组相比，诊断为CG的患者中淋巴管内组织细胞增多症和淋巴管扩张更为常见（54%对15%，P = 0.03；92%对23%，P < 0.01）。CD68+ M1巨噬细胞引起的TH1免疫反应导致CG。巨噬细胞的淋巴管周围聚集和淋巴管内组织细胞增多症是诊断的重要病理线索。
