Department of Dermatology, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Laboratory of Dermatology, Clinical Institute of Inflammation and Immunology, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Front Immunol. 2023 Feb 10;14:1121380. doi: 10.3389/fimmu.2023.1121380. eCollection 2023.
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.
与多发性骨髓瘤(MM)相关的皮肤水疱性发疹较为罕见。虽然大疱的形成主要是由皮肤中的副蛋白淀粉样物质沉积所驱动,但自身免疫也可能发挥作用。在本研究中,我们报告了一例 MM 患者出现水疱的不寻常病例,其表现为松弛性和紧张性水疱和大疱。直接免疫荧光显示 IgA 自身抗体在基底膜带(BMZ)和表皮细胞间隙沉积,呈现出非典型的自身抗体沉积模式。患者病情进展迅速,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(AIBD)进行了文献回顾,发现了 17 例先前报道的病例。与本病例一起,皮肤褶皱处的皮肤受累经常被报道,而黏膜几乎不受影响。半数病例观察到 IgA 天疱疮,具有一致的 IgA 单克隆性。5 例患者的皮肤出现非典型自身抗体沉积模式;这些患者的预后似乎比其他患者差。我们旨在提高对与 MM 或其前体相关的 AIBD 的认识。