Autoimmune bullous diseases:insights into diagnosis and disease management

Autoimmune blistering diseases (AIBDs) are a group of rare and chronic skin and mucosal disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques. MMP is the appropriate term used for pemphigoid diseases with predominant mucosal involvement. One or more mucosal sites can be affected and new lesions on other mucosal sites can develop later during the disease course. Therefore, it is important that patients are monitored for the development of new symptoms. The diagnosis is made based on a biopsy for direct immunofluorescence microscopy combined with serum for indirect immunofluorescence microscopy. Demonstration of antibodies against laminin 332 is important given the possible association between laminin 332 and a malignancy. A multidisciplinary approach in specialized centers is essential in the treatment and monitoring of MMP. In general, the management of AIBDs is based on the use of unspecific systemic immunosuppressive and immunomodulatory therapy with varying results in effectiveness. The introduction of rituximab led to a revolutionary step in the treatment of pemphigus vulgaris. In addition, the administration of additional maintenance infusions of rituximab at month 6 and 12 seems to be beneficial in preventing relapses. In addition to the clinical effectiveness, rituximab showed improvement in the quality of life of patients with pemphigoid and pemphigus. Furthermore, nomacopan, a dual inhibitor of complement C5 and leukotriene B4 (LTB4), appears to be safe and effective for the treatment of bullous and nonbulleus pemphigoid. In contrast, Apremilast, a PDE4 inhibitor, seems not to be effective in the treatment of bullous and nobulleus pemphigoid