淋巴瘤样丘疹病22例临床病理分析

Lymphomatoid papulosis： a clinicopathologic study of 22 cases

目的分析淋巴瘤样丘疹病（LyP）临床表现、组织病理学特点、病程进展及治疗反应。方法回顾性分析2010年6月至2015年3月中国医学科学院北京协和医学院皮肤病研究所确诊的22例LyP患者的临床病理资料。结果22例LyP患者年龄7～83岁，平均39岁，男女比例1∶1。好发部位依次为躯干、四肢、头面部，多数皮损表现为反复发作的红斑、丘疹、结节、溃疡、坏死、结痂，部分愈合后遗留色素沉着或萎缩性瘢痕。组织病理学方面，A型12例，B型1例，C型1例，D型3例，E型4例，B和C型混合型1例。1例确诊之前有原发皮肤间变大细胞淋巴瘤的病史。15例随访患者中，11例经过治疗，治疗方案有口服糖皮质激素、甲氨蝶呤、雷公藤，肌注干扰素，光疗，外用糖皮质激素等。随访时间平均22个月（1～54个月），随访期内所有患者均存活。结论LyP是一种低度恶性的T细胞淋巴瘤，临床呈良性经过，但组织学呈恶性表现，不需要多药联合化疗，预后较好。

ObjectiveTo investigate the clinical presentation, histopathological features, progression, and treatment of lymphomatoid papulosis （LyP）.MethodsA retrospective review was performed on clinicopathological data of 22 patients diagnosed with LyP from June 2010 to March 2015 in Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College.ResultThe mean age of the 22 LyP patients was 39 years （range： 7-83 years）. The male-to-female ratio was 1∶1. The areas predominantly affected were the trunks, followed by limbs and face. Most lesions presented as recurrent eruption of erythema, papule, nodules, ulcer, necrosis and crusting. Some of them leaved hyperpigmentation or atrophic scars on healing. Histopathologically, LyP were devided into types A, B, C, D and E, with 12, 1, 1, 3, and 4 cases in each type, respectively, and one case of mixed type B and C. One of the patients was also diagnosed with primary cutaneous anaplastic large-cell lymphoma besides the diagnosis of LyP. Among the 15 patients with follow-up information available, 11 patients were treated with regimens including oral corticosteroids, methotrexate, tripchlorolide, intramuscular injection of interferon, phototherapy, and topical corticosteroids. The mean follow-up time was 22（1-54）months.All the patients were alive at the end of the follow-up period.ConclusionsLyP is a low-grade malignant T-cell lymphoma with a benign clinical course but histologically malignant features. Multi-agent chemotherapy is unnecessary. Patients with LyP are likely to have an favorable prognosis.