Department of Dermatology, National Skin Centre, Singapore.
Clin Exp Dermatol. 2012 Mar;37(2):118-21. doi: 10.1111/j.1365-2230.2011.04124.x. Epub 2011 Jul 25.
Lymphomatoid papulosis (LyP) is a low-grade cutaneous lymphoma, which lies within the spectrum of primary cutaneous CD30-positive lymphoproliferative disorders. Around 10-20% of LyP cases are associated with a second lymphoma.
To analyse a cohort of Asian patients with LyP, diagnosed from 1987 to 2007 at the National Skin Centre (NSC), Singapore, in terms of epidemiology, treatment and association with a second lymphoma.
Patients were identified through the NSC clinical and histological databases.
During this period, 13 patients were diagnosed with LyP based on clinicopathological criteria. The mean age at diagnosis was 41 years, the male : female ratio was 2.3 : 1, and 92% of the patients were Chinese. Recurrent papulonecrotic lesions were present for a mean of 3 years before diagnosis. Treatment of LyP comprised monotherapy (n = 4) or combination therapy (n = 9) using corticosteroids, oral antibiotics, methotrexate and/or phototherapy. Mean duration of follow-up was 6.4 years. Eight patients (61.5%) were diagnosed with a second lymphoma, either before (n = 2), concurrently with (n = 1) or after (n = 5) the diagnosis of LyP. Mycosis fungoides (MF) was the commonest lymphoma (78%, n = 7), followed by primary cutaneous anaplastic large-cell lymphoma (12%, n = 2). There was one death (mortality rate 7.7%), which occurred in a patient who had developed stage IIA MF after LyP, which subsequently progressed to systemic T-cell lymphoma.
LyP is a chronic, relapsing disease with considerable morbidity, but an overall good prognosis. A strikingly large proportion of our Asian patients (61.5%) had a second lymphoma, compared with previous studies. This emphasizes the importance of regular lifetime surveillance for associated lymphomas in all patients with LyP.
蕈样肉芽肿（LyP）是一种低度皮肤淋巴瘤，属于原发性皮肤 CD30 阳性淋巴增生性疾病谱。大约 10-20%的 LyP 病例与第二种淋巴瘤相关。
分析 1987 年至 2007 年在新加坡国家皮肤中心（NSC）诊断的 LyP 亚洲患者队列，从流行病学、治疗和与第二种淋巴瘤的关联方面进行分析。
通过 NSC 临床和组织学数据库确定患者。
在此期间，根据临床病理标准诊断了 13 例 LyP 患者。诊断时的平均年龄为 41 岁，男女比例为 2.3:1，92%的患者为华人。复发性丘疹坏死性皮损在诊断前平均存在 3 年。LyP 的治疗包括单一疗法（n = 4）或联合疗法（n = 9），使用皮质类固醇、口服抗生素、甲氨蝶呤和/或光疗。平均随访时间为 6.4 年。8 例患者（61.5%）在诊断 LyP 之前（n = 2）、同时（n = 1）或之后（n = 5）诊断出第二种淋巴瘤。蕈样真菌病（MF）是最常见的淋巴瘤（78%，n = 7），其次是原发性皮肤间变性大细胞淋巴瘤（12%，n = 2）。有一例死亡（死亡率 7.7%），发生在 LyP 后发展为 IIA 期 MF 并随后进展为系统性 T 细胞淋巴瘤的患者。
LyP 是一种慢性、复发性疾病，发病率较高，但总体预后良好。与之前的研究相比，我们的亚洲患者中有相当大比例（61.5%）患有第二种淋巴瘤。这强调了在所有 LyP 患者中定期进行终生监测以发现相关淋巴瘤的重要性。
