Boh E, Roberts L J, Lieu T S, Gammon W R, Sontheimer R D
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas 75235-9069.
J Am Acad Dermatol. 1990 Apr;22(4):587-93. doi: 10.1016/0190-9622(90)70077-u.
In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded DNA ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. Patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.
在大多数发生于系统性红斑狼疮患者的获得性大疱性表皮松解症病例中，系统性红斑狼疮的诊断在水疱形成之前就已确立。我们观察到3例有充分记录的获得性大疱性表皮松解症患者，该病在系统性红斑狼疮发病前数年就已出现。1例患者在获得性大疱性表皮松解症确诊时就已产生抗U1RNP自身抗体，且所有5例患者在疾病的系统性红斑狼疮阶段均产生了这种抗体。此外，在所有5例合并系统性红斑狼疮的获得性大疱性表皮松解症病例中，最终均出现了抗双链DNA抗体，4例患者发展为重症系统性红斑狼疮和狼疮性肾炎。对另外15例无明显系统性红斑狼疮的获得性大疱性表皮松解症患者的血清进行了系统性红斑狼疮相关自身抗体分析。发现4例患者至少有一种此类自身抗体。这些发现进一步证明了获得性大疱性表皮松解症与系统性红斑狼疮之间的关联，并表明出现获得性大疱性表皮松解症的系统性红斑狼疮患者可能代表狼疮的一个亚组，使患者发生更严重全身性疾病的风险增加。出现获得性大疱性表皮松解症的患者，尤其是黑人或西班牙裔患者，应监测是否会发展为可能危及生命的系统性红斑狼疮。
