Barnadas Maria A, Gelpí Carmen, Martínez Laura, Curell Román, Martino Rodrigo, Alomar Agustín
Department of Dermatology, Hospital Sta Creu i St Pau, Barcelona, Spain.
J Cutan Pathol. 2010 Nov;37(11):1174-7. doi: 10.1111/j.1600-0560.2009.01383.x.
We present the case of a patient with IgA paraprotein who developed hemorrhagic subepidermal vesicles and bullae with numerous neutrophils. Direct immunofluorescence test (DIF) showed weak deposits of IgA lambda paraprotein at the dermal-epidermal junction and at the intercellular level in the basal layer of the epidermis, and stronger deposits in a perivascular and diffuse pattern in the dermis. Indirect immunofluorescence (IIF) test revealed the presence of circulating IgA lambda antibodies reacting with the intercellular space of monkey and guinea pig esophagus and human skin. A blood test revealed an IgA lambda paraprotein and multiple myeloma stage I(0) was diagnosed in a later hematological study. Dapsone was prescribed and cutaneous lesions improved. This is the second report of subepidermal vesicles and bullae with dermal deposits of IgA paraprotein appearing prior to diagnosis of an IgA multiple myeloma, and it is a unique case with circulating IgA lambda antibodies reacting with the intercellular space of epithelia.
我们报告了一例患有IgA副蛋白的患者,该患者出现了伴有大量中性粒细胞的出血性表皮下水疱和大疱。直接免疫荧光试验(DIF)显示,在真皮-表皮交界处以及表皮基底层的细胞间水平有IgA λ副蛋白的弱阳性沉积,在真皮中呈血管周围和弥漫性较强的沉积。间接免疫荧光(IIF)试验显示存在与猴和豚鼠食管以及人皮肤的细胞间间隙发生反应的循环IgA λ抗体。血液检查发现了IgA λ副蛋白,随后的血液学研究诊断为多发性骨髓瘤I期(0)。给予氨苯砜治疗后,皮肤病变有所改善。这是第二例在IgA多发性骨髓瘤诊断之前出现伴有IgA副蛋白真皮沉积的表皮下水疱和大疱的报告,也是一例循环IgA λ抗体与上皮细胞间间隙发生反应的独特病例。