Calugareanu A, Cordoliani F, Battistella M, Vignon-Pennamen M-D, Lepelletier C, Bagot M, Bouaziz J-D, Auffranc J-C, Jachiet M, Petit A
Service de dermatologie, université Paris-Diderot-Paris VII, Sorbonne-Paris-Cité, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Anatomopathologie, hôpital Saint-Louis, AP-HP, Paris, France.
Ann Dermatol Venereol. 2020 Sep;147(8-9):552-557. doi: 10.1016/j.annder.2020.03.003. Epub 2020 Apr 22.
Cutaneous plasmacytosis is a rare skin condition first described in 1976 and it is seen mainly in patients of Asian descent. Patients usually present with multiple reddish-brown macules and nodules chiefly on the trunk and face, with clusters of well-differentiated plasma cells in the dermis. The aetiopathogenesis and nosological features of this entity remain obscure. We report herein a case of cutaneous plasmacytosis in a European middle-aged woman with presence of Darier's sign.
A 56-year-old woman of European descent presented with asymptomatic hyperpigmented patches affecting the dorsal aspect of her trunk for at least two years. Darier's sign was present in some episodes. Cutaneous biopsy showed a moderately dense interstitial and perivascular infiltrate containing numerous well-differentiated mature plasma cells affecting the entire dermal surface. Kappa and lambda immunochemistry demonstrated polyclonal plasma cell infiltrates with absence of light-chain restriction. Immunohistochemical examination was negative for HHV-8 and Treponema pallidum spirochetes. Laboratory findings revealed hypergammaglobulinaemia with no monoclonal bands being detected on immunofixation. A diagnosis of cutaneous plasmacytosis was made. In the absence of systemic involvement initial management consisted of clinical surveillance.
The characteristic clinico-pathological features of CP allowed diagnosis of this skin condition in our patient, although it is very rarely reported in patients of European descent. The main differential diagnoses were ruled out, namely plasmacytic infiltrates related to infections and marginal B-cell lymphoma.
皮肤浆细胞增多症是一种罕见的皮肤病，于1976年首次被描述，主要见于亚洲血统的患者。患者通常表现为主要位于躯干和面部的多个红棕色斑疹和结节，真皮中有分化良好的浆细胞簇。该疾病的病因发病机制和分类学特征仍不明确。我们在此报告一例欧洲中年女性的皮肤浆细胞增多症病例，该患者存在达里埃氏征。
一名56岁的欧洲血统女性，其躯干背部出现无症状色素沉着斑至少两年。在某些发作中存在达里埃氏征。皮肤活检显示中度致密的间质和血管周围浸润，其中含有大量影响整个真皮表面的分化良好的成熟浆细胞。κ和λ免疫化学显示多克隆浆细胞浸润，无轻链限制。免疫组织化学检查对HHV-8和梅毒螺旋体呈阴性。实验室检查结果显示高球蛋白血症，免疫固定未检测到单克隆条带。诊断为皮肤浆细胞增多症。由于无全身受累，初始治疗包括临床监测。
尽管在欧洲血统患者中很少报道，但CP的特征性临床病理特征使我们能够对该患者的这种皮肤病进行诊断。主要的鉴别诊断被排除，即与感染相关的浆细胞浸润和边缘B细胞淋巴瘤。
