Schoenlaub P, Lipsker D, Massard G, Christmann D, Grosshans E
Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg.
Ann Dermatol Venereol. 1997;124(3):228-32.
We report two patients in whom a slowly growing erythematous thoracic lesion led to the diagnosis of an underlying plasmocytoma. After the treatment of the latter, the cutaneous lesions disappeared, strongly suggesting a link between the two manifestations.
The two male patients, aged respectively 66 and 73 years old, had erythematous thoracic plaques. In both cases, extensive laboratory work-up and a histological examination of a cutaneous biopsy did not allow a precise diagnosis. The two patients had a solitary bony plasmocytoma located beneath the cutaneous plaques. The plasmocytomas were discovered respectively 2 and 4 years after the first cutaneous manifestations and were associated to histological non-specific lymph node hyperplasia. Treatment of the plasmocytoma led to the disappearance of skin lesions in both patients.
In both cases, an erythematous scleroderma-like plaque associated to palpable peripheral and mediastinal lymph nodes, was located over a solitary bony plasmocytoma. Treatment of the plasmocytoma led to the disappearance of the cutaneous lesions, strongly suggesting a link between the two manifestations. Such an association has not been previously reported. We think this is a new entity, characterised by a scleroderma-like cutaneous plaque overlying a solitary bony plasmocytoma and associated to superficial and deep lymph node hyperplasia. We suggest to call it "plasmocytoma associated cutaneous lymph node syndrome". Its relationship to the POEMS syndrome and scleromyxedema are discussed. The pathophysiology remains completely unknown; the syndrome regresses after the treatment of the plasmocytoma.
我们报告两例患者，其胸部缓慢生长的红斑性病变最终确诊为潜在的浆细胞瘤。在治疗浆细胞瘤后，皮肤病变消失，强烈提示这两种表现之间存在关联。
两名男性患者，年龄分别为66岁和73岁，均有胸部红斑性斑块。在这两例中，广泛的实验室检查和皮肤活检的组织学检查均未能明确诊断。两名患者在皮肤斑块下方均有孤立性骨浆细胞瘤。浆细胞瘤分别在首次皮肤表现出现2年和4年后被发现，并伴有组织学上非特异性的淋巴结增生。浆细胞瘤的治疗使两名患者的皮肤病变均消失。
在这两例中，与可触及的外周和纵隔淋巴结相关的红斑性硬皮病样斑块均位于孤立性骨浆细胞瘤上方。浆细胞瘤的治疗导致皮肤病变消失，强烈提示这两种表现之间存在关联。此前尚未有此类关联的报道。我们认为这是一种新的疾病实体，其特征为孤立性骨浆细胞瘤上方的硬皮病样皮肤斑块，并伴有浅表和深部淋巴结增生。我们建议将其称为“浆细胞瘤相关皮肤淋巴结综合征”。文中讨论了它与POEMS综合征和硬化性黏液水肿的关系。其病理生理学仍完全未知；该综合征在浆细胞瘤治疗后消退。
