Objective To investigate the etiology, diagnosis and differential diagnosis, treatment and prognosis of lymphoma papulosis for improving the cognition on this disease. Methods The clinical, histopathological and immunofluorescence data in one case of the lymphoma papulosis were analyzed and the relevant literature was analyzed. Results The Patient with lymphoma papulosis showed erythema, papules, blisters, scabs with itch happened repeatedly in entire body over one month. Dermatologist check: visible diffuse distribution of erythema, papules, and blisters, all of which had the size of rice to the size of soybean, on the trunk and limbs; partial skin had necrosis, and scabs. The ulcer, which had the size of soybean to the size of coin, could be seen on the scrotum and penis skin. The whole body had no obvious pustules, and each joint and toenail had no obvious abnormity. Skin tissue pathological examination: degeneration and necrosis of epidermis, formation of subepidermal blisters, intradermal hemangiectasis, fibrinoid degeneration of vascular wall, obvious inflammatory cell infiltration around the blood vessels, and obvious abnormity cells and nuclear mitosis could be seen. Immunofluorescence examination: IgG (-), IgM (-), IgA (-), C3 (-). The acid fast stain was negative. Immunohistochemical examination: intradermal infiltrating cells expressed CD3 (+), CD4 (-), CD8 (+), CD20 (-), CD30 (+), CD56 (-), and Ki67 (+>50%). T lymphoma cloned gene rearrangement (+). Diagnosis: C lymphoma papulosis. Conclusion The lymphomatoid papulosis (LyP) is an inert cutaneous lymphoma that features chronic relapsing, self-healing skin papules, necrosis, nodules, and clinical benign while appearing malignance in histopathology; and it belongs to skin CD30 (+) lymphoproliferative disease. Its exact pathogenesis is not yet clear. The treatment can only alleviate the symptoms, but cannot prevent the relapse of the disease. At present, there is no effective treatment in preventing the secondary lymphoma, and long-term follow-up monitoring and treatment of the secondary lymphoma are needed.%目的：探讨淋巴瘤样丘疹病的病因、诊断和鉴别诊断、治疗及预后，以提高对该病的认识。方法对1例C型淋巴瘤样丘疹病的临床资料、组织病理、免疫荧光进行分析，并对相关文献进行分析。结果患者全身反复红斑、丘疹、水疱、结痂伴痒1个月余。皮肤科检查：躯干、四肢可见弥漫分布的米粒至黄豆大小的红斑、丘疹、水疱，部分皮损可见坏死、结痂。阴囊及阴茎部皮肤可见黄豆至钱币大小溃疡。全身未见明显脓疱，各关节及指趾甲未见明显异常。皮肤组织病理检查：表皮变性坏死，表皮下水疱形成，真皮内血管扩张，管壁纤维素样变性，血管周围见明显炎性细胞浸润，可见明显异型细胞及核丝分裂。免疫荧光检查：IgG (－)、IgM (－)、IgA (－)、C3(－)。抗酸染色阴性。免疫组化检查：真皮内浸润细胞表达CD3(+)、CD4(－)、CD8(+)、CD20(－)、CD30(+)、CD56(－)、Ki67(+＞50%)。T淋巴瘤克隆性基因重排（+）。诊断：C型淋巴瘤。结论淋巴瘤样丘疹病（lymphoma papulosis，LyP）是一种慢性复发性、自愈性皮肤丘疹、坏死、结节，临床结果良性、但组织病理上呈恶性表现的惰性皮肤淋巴瘤，属于皮肤CD30（+）淋巴增殖性疾病。其确切的发病机制还不清楚。治疗只能缓解症状，而不能阻止疾病的转归，目前还缺乏有效预防继发淋巴瘤的治疗方法，需长期随访监测并治疗继发性淋巴瘤。
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