Epidermolysis Bullosa Acquisita: The 2019 Update

Abstract

Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Current treatment options rely on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment. Hence, novel treatment options are urgently needed for the care of EBA patients. During the past decade, decisive clinical observations, and frequent use of pre-clinical model systems have tremendously increased our understanding of EBA pathogenesis. Herein, we review all of the aspects of EBA, starting with a detailed description of epidemiology, clinical presentation, diagnosis, and current treatment options. Of note, pattern analysis via direct immunofluorescence microscopy of a perilesional skin lesion and novel serological test systems have significantly facilitated diagnosis of the disease. Next, a state-of the art review of the current understanding of EBA pathogenesis, emerging treatments and future perspectives is provided. Based on pre-clinical model systems, cytokines and kinases are among the most promising therapeutic targets, whereas high doses of IgG (IVIG) and the anti-CD20 antibody rituximab are among the most promising “established” EBA therapeutics. We also aim to raise awareness of EBA, as well as initiate basic and clinical research in this field, to further improve the already improved but still unsatisfactory conditions for those diagnosed with this condition.

Review of autoimmune blistering diseases: the Pemphigoid diseases

“…It is a rare disease affecting about 0.2/million . It can occur at any age though there is a bimodal peak in the 2nd and 7th decade …”

Blistering and scarring confined to the head

“…Direct immunofluorescence studies of perilesional skin show linear deposits of IgG, complement and, more rarely, IgA. A u-serrated pattern of antibody deposition is characteristic of EBA [2,6], whereas an n-serrated pattern is typically seen in bullous pemphigoid. While type VII collagen is the main component of the anchoring fibrils in the papillary dermis [1], it is also found in mucous membranes (MM-EBA) ( Table 1).…”

Vitamin D in autoimmune bullous disease

“…As in the case of pemphigus, the pemphigoid diseases can be controlled medically by using corticosteroids, high-doses of IVIG, rituximab, plasmapheresis, and immunoadsorption Koga et al, 2019).…”