Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology
An. bras. dermatol
; 94(2,supl.1): 33-47, Mar.-Apr. 2019. graf
Article
in English
| LILACS
| ID: biblio-1011091
Responsible library:
BR1.1
ABSTRACT
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.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Epidermolysis Bullosa Acquisita
/
Pemphigoid, Benign Mucous Membrane
/
Pemphigoid, Bullous
/
Consensus
Type of study:
Diagnostic study
/
Practice guideline
/
Prognostic study
Limits:
Humans
Country/Region as subject:
South America
/
Brazil
Language:
English
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2019
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal de São Paulo/BR
/
Universidade de São Paulo/BR
/
Universidade do Estado do Rio de Janeiro/BR