Kindler Syndrome

Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn's disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.