Pech J H, Moreau-Cabarrot A, Oksman F, Bedane C, Bernard P, Bazex J
Service de Dermatologie, CHU Purpan, Toulouse.
Ann Dermatol Venereol. 1997;124(4):325-8.
Waldenström macroglobulinemia is a rare hematologic disorder with characteristic malignant plasma cell proliferation associated with the secretion of a IgM monoclonal immunoglobulin which is the cause of most of the clinical manifestations. Skin involvement is exceptional. We report a new case of Waldenström macroglobulinemia discovered in a patient who developed specific skin lesions with monoclonal immunoglobulin deposits.
A 50-year-old woman developed spontaneously painful infiltrated erythematopapular plaques over the extension aspects of the limbs. There were no other clinical manifestations. The patient had a kappa IgM monoclonal gammapathy. The diagnosis of Waldenström macroglobulinemia was proposed after the discovery of voluminous intra-abdominal adenopathies and was confirmed by immunohistochemistry of the biopsies, indirect immunofluorescence evidenced reactivity against the epidermal basal membrane. Chemotherapy led to successful regression of the skin lesions, the tumoral mass and circulating IgM.
This is the second reported case of Waldenström macroglobulinemia with anti-basal membrane monoclonal immunoglobulin. This IgM would recognize an 82 kD antigen on the dermal side of the dermo-epidermal junction situated in the anchoring fibres of the lamina densa, a zone classically involved in acquired bullous epidermolysis.
华氏巨球蛋白血症是一种罕见的血液系统疾病，其特征为恶性浆细胞增殖，并伴有IgM单克隆免疫球蛋白的分泌，这是大多数临床表现的病因。皮肤受累情况较为罕见。我们报告了一例新发现的华氏巨球蛋白血症病例，该患者出现了伴有单克隆免疫球蛋白沉积的特异性皮肤病变。
一名50岁女性在四肢伸侧自发出现疼痛性浸润性红斑丘疹斑块。无其他临床表现。患者存在κ型IgM单克隆丙种球蛋白病。在发现大量腹腔淋巴结肿大后，提出了华氏巨球蛋白血症的诊断，并通过活检的免疫组织化学得以证实，间接免疫荧光显示对表皮基底膜有反应性。化疗使皮肤病变、肿瘤肿块和循环IgM成功消退。
这是第二例报告的伴有抗基底膜单克隆免疫球蛋白的华氏巨球蛋白血症病例。这种IgM可识别位于致密板锚定纤维中的真皮表皮交界处真皮侧的一种82 kD抗原，该区域是经典的获得性大疱性表皮松解症受累部位。
