Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune blistering disease in which blisters on skin as well as on mucous membranes develop due to binding of IgG autoantibodies to type VII collagen (structural component of anchoring fibrils) in upper dermis. EBA has two major clinical subtypes – mechanubullous and inflammatory variants. Mechanobullous variant present with skin fragility, blisters, scarring, milia, dystrophic changes on trauma-prone areas and can resemble features seen in DEB, inflammatory variants resemble other AIBD as BP-like, MMP-like and Brunsting Perry pemphigoid like as well as LABD like disease which is most common in children. Diagnosis is based on clinical picture, histology and direct immunofluorescence which can be very similar to BP. In salt split skin immunofluorescence finding in patients with EBA deposits are found on the dermal side of the blister. Comercial anti col VII ELISA kits are available. Therapeutically, the disease can be very resistant. Treatment options are systemic corticosteroids, IVIg, colhicin, dapsone, rituximab.

  • Specific challenges associated with the recognition of the condition

EBA can be misdiagnosed as BP according histopathology and DIF if salt splited skin is not performed. As it usually does not respond well to the therapy with corticosteroids, additional laboratory investigations are performed. So delay of diagnosis can be 6 months or more.

  • . Specific challengesassociated with the diagnosis

Diagnosis of EBA is based on the combination of careful clinical examination of skin and all mucous membranes, and histopathology, direct and indirect immunofluorescence of affected mucous membrane and healthy skin. Indirect immunofluorescence (on salt splitted skin). Comercial ELISA as well as immunoblot are available and useful for final diagnosis.

  • Specific challenges associated with the treatment

EBA can be a therapy resistant disease. Although it is known that oral corticosteroids are not very effective in this disease, patiens are often treated with CS as a first line treatment. According to the literature, colchicine or dapsone could be treatment of choice. If this therapy does not give results, immunosuppressants, IVIg or rituximab should be tried.

  • Specific challenges associated with care of these patients over their lifespan – Quality of life issues – Gaps across the care continuum

Optimal management of EBA patients require a multidisciplinary approach by highly specialized dermatologists, as well as specialists of other specialities as well as general practitioners. Specialists who can be involved in the management of corticosteroid- side effects are rheumatologists, endocrinologists and ophtalmologists. Nurses are also involved in the therapy. Nursing staff play also a major role in patients’ educational programs. Many patients complain of long-lasting symptoms or psychological troubles, that frequently need the intervention of psychologists.

Prevalence :  Prevalence unknown

 

 

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