Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa
acquisita are subepidermal autoimmune blistering diseases whose antigenic target
is located at the basement membrane zone. Mucous membrane pemphigoid and
epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement,
leading to respiratory, ocular and/or digestive sequelae with important
morbidity. For each of these dermatoses, a literature review covering all
therapeutic options was performed. A flowchart, based on the experience and
joint discussion among the authors of this consensus, was constructed to provide
treatment orientation for these diseases in Brazil. In summary, in the
localized, low-risk or non-severe forms, drugs that have immunomodulatory action
such as dapsone, doxycycline among others may be a therapeutic option. Topical
treatment with corticosteroids or immunomodulators may also be used. Systemic
corticosteroid therapy continues to be the treatment of choice for severe forms,
especially those involving ocular, laryngeal-pharyngeal and/or esophageal
mucosal involvement, as may occur in mucous membrane pemphigoid and
epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant
alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an
alternative that, while expensive, may be used. Immunobiological drugs such as
rituximab are promising drugs in this area. Omalizumab has been used in bullous
pemphigoid.