与多发性骨髓瘤(MM)相关的皮肤水疱性皮疹很少见。虽然水泡的发展主要是由皮肤中的副蛋白的淀粉样沉积物驱动的,自身免疫可能发挥作用。在这项研究中,我们报告了一例MM患者出现水泡的不寻常病例,表现为松弛和紧张的囊泡和大疱。直接免疫荧光显示IgA自身抗体沉积在基底膜区(BMZ)和表皮的细胞间隙,显示非典型自身抗体沉积模式。患者显示疾病进展迅速,并在随访期间死亡。我们对与MM或其前体相关的自身免疫性大疱性疾病(AIBDs)进行了文献综述,发现了17例以前报道的病例。连同本案,经常报道皮肤褶皱的皮肤受累,粘膜几乎没有受到影响。IgA天疱疮,具有一致的IgA单克隆性,在一半的病例中观察到。五名患者在皮肤中表现出非典型的自身抗体沉积模式;这些患者的预后似乎比其他患者差。我们的目标是增加我们对与MM或其前体相关的AIBD的理解。
Cutaneous vesiculobullous eruptions associated with multiple myeloma (MM) are rare. Although the development of blisters is mostly driven by amyloid deposits of paraproteins in the skin, autoimmunity may play a role. In this study, we report an unusual
case of an MM patient with blisters who presented with both flaccid and tense vesicles and bullae. Direct immunofluorescence revealed IgA autoantibody deposits in the basement membrane zone (BMZ) and intercellular space of the epidermis, which displayed an atypical autoantibody deposition pattern. The patient showed rapid disease progression and died during follow-up. We performed a literature review of autoimmune bullous diseases (AIBDs) associated with MM or its precursors and found 17 previously reported cases. Together with the present
case, cutaneous involvement of the skin folds was frequently reported, and mucous membranes were barely affected. IgA pemphigus, with consistent IgA monoclonality, was observed in half of the cases. Five patients displayed atypical autoantibody deposition patterns in the skin; the prognosis of these patients appeared to be poorer than that of other patients. We aim to increase our understanding of AIBDs associated with MM or its precursors.