Malissen N, Fabre C, Joujoux J-M, Bourquard P, Dandurand M, Marque M, Stoebner P, Meunier L
Service de dermatologie, CHU de Nîmes, place du Pr R. Debré, 30029 Nîmes cedex 9, France.
Service de dermatologie, CHU de Nîmes, place du Pr R. Debré, 30029 Nîmes cedex 9, France.
Ann Dermatol Venereol. 2014 May;141(5):364-8. doi: 10.1016/j.annder.2014.01.003. Epub 2014 Feb 17.
Primary cutaneous plasmacytoma is a rare form of cutaneous B-cell lymphoma.
A 51 year-old male with an unremarkable history gradually presented erythematous papulonodular lesions that had appeared gradually over the whole body throughout a two-year period and showing histologic and immunohistochemical features of cutaneous plasmacytoma. Staging investigations confirmed the primary character of the disease, and because of this and the absence of functional impairment, we opted for therapeutic abstention. No progression was noted after 4 years of regular monitoring.
Primary cutaneous plasmacytoma (PCP) is characterized by clonal proliferation of plasma cells in skin. Multiple PCPs are extremely rare and to date have been treated in most cases by chemotherapy, either with or without radiotherapy. The prognosis is poor, with 2-year survival of only 25%. The present case is original, being the only one to our knowledge in which therapeutic abstention was followed by a lack of progression after 4 years of regular follow-up. Consequently, certain indolent forms of PCP do not warrant automatic institution of chemotherapy.
原发性皮肤浆细胞瘤是皮肤B细胞淋巴瘤的一种罕见形式。
一名51岁男性,既往史无特殊,逐渐出现红斑丘疹结节性皮损,在两年时间里全身逐渐出现,组织学和免疫组化表现为皮肤浆细胞瘤。分期检查证实了该疾病的原发性,鉴于此且无功能损害,我们选择暂不治疗。定期监测4年后未见病情进展。
原发性皮肤浆细胞瘤(PCP)的特征是皮肤中浆细胞的克隆性增殖。多发性PCP极为罕见,迄今为止,大多数病例采用化疗治疗,联合或不联合放疗。预后较差,2年生存率仅为25%。本病例具有独特性,据我们所知,这是唯一一例在定期随访4年后未进行治疗且病情未进展的病例。因此,某些惰性形式的PCP不一定需要立即进行化疗。