Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

Abstract

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.

Manifestação Oral De Lesão Penfigóide - Relato De Caso E Revisão Da Literatura

“…Quando ocorre manifestações cutâneas geralmente essas lesões são presentes na face, couro cabeludo, pescoço e a parte superior do peito. O que normalmente aparecem como placas eritematosas, formação bolhas e erosões recorrentes 20,21 .…”

Epidermolysis bullosa acquisita treated with rituximab

“…EBA is a rare acquired, mucocutaneous, subepidermal, autoimmune blistering disease, which results in vesicle and bullae formation on the skin and erosions on the mucous membranes. [1][2][3][4][5] EBA can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. [1][2][3] It usually appears in adulthood (with a median age of 50 years), with no known predilection to sex.…”

“…[1][2][3][4][5] EBA can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. [1][2][3] It usually appears in adulthood (with a median age of 50 years), with no known predilection to sex. [1][2][3] Clinically, it can present with numerous phenotypes, though the most common are the mechanobullous and inflammatory variants; EBA includes various distinct clinical manifestations resembling bullous pemphigoid, Brunsting-Perry pemphigoid or cicatricial pemphigoid.…”

“…[1][2][3] It usually appears in adulthood (with a median age of 50 years), with no known predilection to sex. [1][2][3] Clinically, it can present with numerous phenotypes, though the most common are the mechanobullous and inflammatory variants; EBA includes various distinct clinical manifestations resembling bullous pemphigoid, Brunsting-Perry pemphigoid or cicatricial pemphigoid. [1][2][3][4] There is an association with HLA-DR2 and the patients have autoantibodies against collagen VII, an integral component of anchoring fibrils responsible for attaching the dermis to the epidermis.…”

Mucous membrane pemphigoid in a patient with chronic hepatitis B virus infection

“…Moreover, the treatment regarding systemic immunosuppressants may need to be assessed thoroughly with the specific underlying chronic condition. [ 3 ] Here we present a case with recurrent swellings of the neck, cheeks, eyelids, and lips, followed by the formation of hemorrhagic bullae with scar over oral mucosa and skin. The patient was diagnosed with mucous membrane pemphigoid 2 years after the onset of the initial symptoms.…”