Andrès P, Lepagney M L, Bureau B, Litoux P, Dréno B
Department of Dermatology, Hôtel-Dieu Hospital, Nantes, France.
Int J Dermatol. 1997 Aug;36(8):582-6. doi: 10.1046/j.1365-4362.1997.00274.x.
A retrospective clinical, histologic, and immunohistochemical study was performed in 37 cases of isolated primary cutaneous lymphoma (PCL) (22 B and 15 T phenotype). Patients with epidermotrophic infiltrate (mycosis fungoides and Sézary syndrome) were excluded.
Patients with PCL were selected according to strict criteria: isolated cutaneous involvement for at least 6 months and a negative exhaustive study of possible spread. Lesions were either limited to a single cutaneous region or were disseminated, involving at least two nonadjacent regions. The diagnosis was confirmed histologically, and an immunohistochemical study was performed.
On the basis of the new Willemze classification for prognostic criteria, this study showed similarities between lymphomas of B and T phenotype in clinical features, therapeutic response, course, and overall prognosis. The clinical lesion was usually an erythematous nodule associated, or not, with an infiltrated layer and generally limited to a single cutaneous region. PCLs were highly sensitive to nonaggressive treatment, showing complete or more than 50% partial remission in all cases.
The overall prognosis for these lymphomas was good, even for disseminated cutaneous forms. Patient survival at 48 months was 78% for T and 89% for B phenotype. In the latter group, the prognosis was comparable for CD30+ and CD30- T lymphomas; however, the course of PCL involved frequent cutaneous relapses, particularly with the disseminated forms, raising the problem of adjuvant treatment after complete remission was obtained. Extracutaneous involvement was rare, but always indicative of poor prognosis.
对37例孤立性原发性皮肤淋巴瘤（PCL）（22例B表型和15例T表型）进行了回顾性临床、组织学和免疫组化研究。排除有亲表皮浸润的患者（蕈样肉芽肿和Sezary综合征）。
根据严格标准选择PCL患者：皮肤孤立受累至少6个月，且对可能的播散进行全面检查为阴性。病变要么局限于单个皮肤区域，要么为播散性，累及至少两个不相邻区域。通过组织学确诊，并进行免疫组化研究。
根据新的Willemze预后标准分类，本研究显示B表型和T表型淋巴瘤在临床特征、治疗反应、病程和总体预后方面具有相似性。临床病变通常为红斑结节，可伴有或不伴有浸润层，一般局限于单个皮肤区域。PCL对非侵袭性治疗高度敏感，所有病例均显示完全缓解或超过50%的部分缓解。
这些淋巴瘤的总体预后良好，即使是播散性皮肤型也是如此。T表型患者48个月生存率为78%，B表型为89%。在后一组中，CD30+和CD30-T淋巴瘤的预后相当；然而，PCL病程中皮肤复发频繁，尤其是播散性类型，这就提出了在获得完全缓解后辅助治疗的问题。皮肤外受累很少见，但总是提示预后不良。
