Management of epidermolysis bullosa acquisita

By all accounts, epidermolysis bullosa acquisita (EBA) is a very rare chronic subepidermal blistering disease which involves the skin and mucous membranes. The disease can appear in childhood, but has a wide age range with no sex, racial or ethnic group predilection. There is an absence of family or personal history of any blistering diseases. There is a developing association of the HLA‐DR2 allele and some of these cases can have enhanced susceptibility to bullous SLE. Other disease associations with EBA include inflammatory bowel disease, amyloidosis, myeloma, thyroiditis, diabetes and multiple endocrinopathy syndrome. EBA often presents with skin fragility and blistering that is usually trauma induced, so that the elbows, knees, knuckles, fingers and fingernails tend to be affected. The lesions heal with scarring and milia formation. However, some cases of EBA can have a clinical phenotype, similar to bullous pemphigoid, with widespread inflammatory vesicles and bullae. Other cases may present similarly to cicatricial pemphigoid with involvement of the eyes, mouth and genital areas. The severity of EBA varies, indicating a rather heterogenous nature. Milder cases undoubtedly occur and some of these go into spontaneous remission, but very severe cases can lead to blindness, oesophageal strictures and anal stenosis and be very incapacitating. The diagnosis of EBA can be confirmed by salt‐split immunofluoresence procedures where the BMZ antibodies bind to the dermal side of the split. Ideally, one wishes to identify type 7 collagen by Western blot analysis, ELISA or immunoelectron microscopy. EBA can be a very frustrating and difficult disease to treat. In our experience, EBA can show great heterogeneity in the response to therapy. Some cases appear mild and respond to low dose prednisolone with dapsone, 50–100 mg daily. More moderately severe cases require prednisolone, azathioprine or cyclosporin. There are, undoubtedly, very severe cases which appear to be resistant to all of the above therapies. It is not advised that very high dose oral corticosteroids he administered in EBA, as often this is ineffective and leads to crippling steroid side‐effects. For very resistant or severe cases, the use of oral mycophenolate mofetil can be recommended. Some cases may respond to IV pulses of prednisolone and cyclophosphamide and others, to IVIG infusions. Unfortunately, because of the rarity of the disease, no exact therapeutic policy has been established and each case requires consideration on its merits. Management of cases with severe ocular, oesophageal or genital involvement is best done within a specialized centre which includes facilities for endoscopic techniques for strictures.