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.

Imunoglobulina intravenosa para tratamento de epidermólise bolhosa adquirida grave refratária a terapia imunossupressora convencional / Intravenous immunoglobulin for treatment of severe acquired bullous epidermolysis refractory to conventional immunosuppressive therapy

A epidermólise bolhosa adquirida é doença bolhosa subepidérmica crônica e rara. Geralmente, inicia-se na fase adulta, sendo a etiologia desconhecida, embora vinculada à presença de anticorpos contra o colágeno tipo VII. Há formação de bolhas, espontaneamente ou após trauma, podendo causar complicações graves. O tratamento é desapontador e difícil. Além da terapia convencional com corticoides sistêmicos, recentemente, novas modalidades terapêuticas promissoras estão sendo utilizadas, dentre elas, a imunoglobulina intravenosa. Destaca-se, neste relato, o difícil manejo clínico desta doença, e a melhora importante com a imunoglobulina intravenosa.

Acquired bullous epidermolysis is a chronic and rare bullous subepidermal disease. It usually begins in adulthood and its etiology is unknown although it is associated with antibodies against type VII collagen. There are spontaneous and trauma induced formation of blisters that may cause serious complications. Treatment is disappointing and difficult. Apart from conventional therapy with systemic corticosteroid, new therapeutic modalities such as intravenous immunoglobulin are currently being used. This report highlights the extremely difficult clinical management of this rare disease and the important improvement provided by intravenous immunoglobulin.

Epidermolisis ampollar adquirida y lupus eritematoso sistémico: autoinmunidad mediada por anticuerpos contra colágeno tipo VII / Acquired bullous epidermolysis and systemic lupus erythematosus: autoimmunity mediated by type VII collagen antibodies

La epidermolisis ampollar adquirida (EAA) es una enfermedad autoinmune provocada por autoanticuerpos contra el colágeno tipo VII de la sublámina densa de la dermis. Sus manifestaciones clínicas exigen especial atención para diferenciarla del lupus ampollar (LA), el penfigoide ampollar (PA) y la porfiria cutánea tarda (PCT). La clínica, la histopatología, la inmunofluorescencia directa (IFD) y la inmunofluorescencia indirecta (IFI) con la técnica de salt-split son de gran ayuda para el diagnóstico. El caso de una paciente con EAA y lupus eritematoso sistémico (LES) plantea la discusión sobre las diferencias y semejanzas entre el LA y la EAA. La presencia de mecanismos inmunopatogénicos comunes y la asociación de LES y EAA permitiría suponer que se trata de expresiones clínicas de una misma afección

El espectro clínico e histopatológico de la epidermolisis ampollar adquirida: reporte de tres casos / Clinical and pathologic spectrum of epidermolysis bullosa acquisita: report of three cases

La epidermolisis ampollar adquirida (EAA) es una enfermedad ampollar subepidérmica, crónica, poco frecuente, caracterizada por la presencia de anticuerpos circulantes y/o tisulares tipo IgG dirigidos contra el colágeno tipo VII de las fibrillas de anclaje de la unión dermo-epidérmica. Su aspectro clínico e histopatológico es variable y se han definido tres presentaciones: la forma clásica similar a una porfiria cutánea tarda o a una epidermolisis ampollar distrófica, la forma tipo penfigoide ampollar y la forma tipo penfigoide cicatricial. El diagnóstico definitivo se establece mediante la inmunofluorescencia directa con piel perilesional separada con NaCI 1 molar o mediante la inmunomicroscopía electrónica. En nuestro medio, es una enfermedad poco frecuente o no detectada ya que plantea dificultades diagnósticas con otras enfermedades ampollares. En este estudio presentamos tres casos catalogados como EAA recolectados con la consulta de enfermedades ampollares de nuestro centro, que ilustran la diversidad clínica e histológica de esta entidad

Lúpus eritematoso sistêmico bolhoso / Bullous systemic lupus erythematosus

O lupus eritematoso sistêmico bolhoso é doença subepidérmica rara, com caracteríticas clínicas, histológicas e imunopatológicas distintas. Este trabalho objetiva fazer atualizaçäo de aspectos da etiopatogenia, curso clínico, histopatologia, imunopatologia, diagnóstico diferencial e tratamento da doença. Enfoque especial é dado à imunopatologia

Immunofluorescence in differentiating between bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid of brunsting perry variant

Direct and indirect immunofluorescence are helpful in diagnosing vesicobullous disorders. However, similar deposits along the basement membrane zone are seen in bullous pemphigoid [BP], cicatricial pemphigoid [CP] and epidermolysis bullosa acquisita [EBA]. Immunofluorescence studies were performed in 3 cases of recurrent vesicobullous disorders with atypical clinical features by standard DIF, IIF, split skin techniques and by isopropyl alcohol fixation method. Diagnosis was confirmed by the ability of CP and BP to localise at the roof of split skin, and of EBA antigen to fluoresence the floor of split skin. The diagnosis of EBA and BP was further confirmed by loss of immunofluorescence in BP following the incubation of the substrate in isopropyl alcohol and continued fluorescence inspite of similar treatment in EBA. The fluorescence was seen to persist also in C P. Immunofluorescence using split skin techniques are helpful in differentiating subepidermal blisting disorders like BP, EBA and CP. Isopropyl alcohol fixation is an additional method to help in their differentiation

Epidermólisis ampollar adquirida / Epidemolysis bullosa acquista

Se presenta el caso de una paciente, de sexo femenino, de 55 años con diagnóstico clínico e inmunopatológico de epidermólisis ampollar adquirida, con compromiso extenso de mucosas, afectando hasta tercio superior de esófago. Se efectuó una revisión del tema en sus aspectos epidemiológicos, clínicos, inmunohistológicos, pronósticos y terapéuticos

Immuno-fluorescent study in patients with auto-immune bullous dermatoses

Sixty-seven patients with Auto-Immune Bullous Dermatoses were studied immunologically in alignment with a comparison of 25 apparently healthy age and sex matched control group. Immunfluorescent tests were the main guide for specific diagnosis and classification of these dermatoses. Five important bullous diseases have been reported for the first time in Iraq using both direct and indirect immunofluorescent exams these are juvenile pemphigus, pemphigus herpertiformis, linear IgA disease, epidermolysis bullosa acquistia and linear IgM disease

Epidermolysis bullosa aquisita with basal epidermal cytoplasmic antibodies

A 45-year-old woman with epidermolysis bullosa aquisita is presented. The clinical, histological, and immunopathological features were in keeping with the previous reports of this disease. The patient also had anti-basal cell cytoplasmic antibodies at a significant titer, which is considered an unusual finding associated with this disorder. Treatment with a moderate dose of corticosteroid was effective in controlling the bullous lesions