Imunoglobulina intravenosa para tratamento de epidermólise bolhosa adquirida grave refratária a terapia imunossupressora convencional

Abstract

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. Keywords: Acquired bullous epidermolysis; Immunosuppressive agents; Immunoglobulins, intravenous Resumo: 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 sistêmi-cos, 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. Palavras-chave: Epidermólise bolhosa adquirida; Imunoglobulinas intravenosas; Imunossupressores

Epidermolysis Bullosa: Literature Review

“…As for the acquired form, it was verified that it is a disease that starts in adulthood, regardless of race or gender, however, it usually settles in adulthood (higher incidence at 50 years of age), being related to the HLA-DR2 allele ( MOSQUEIRA, 2010). Accordingly, the study by Silva (2020) revealed that people with this phenotype are more susceptible to the deposit of IgG and C3 in the basement membrane (occasionally IgA and IgM are also present).…”

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

“…IVIG modulates the autoimmune response, reducing and neutralizing antibodies. 117 It is usually administered at 2g/kg/ cycle over 3 to 5 days as monotherapy or in combination with other drugs, such as systemic corticosteroid and dapsone, with good response in patients with recalcitrant disease. 103,117 One long-term study evaluated 10 patients with EBA that were resistant to conventional therapy after IVIG administration, resulting in a satisfactory response in all cases.…”

“…117 It is usually administered at 2g/kg/ cycle over 3 to 5 days as monotherapy or in combination with other drugs, such as systemic corticosteroid and dapsone, with good response in patients with recalcitrant disease. 103,117 One long-term study evaluated 10 patients with EBA that were resistant to conventional therapy after IVIG administration, resulting in a satisfactory response in all cases. 118 Patients received 16-31 IVIG cycles over 30-52 months, allowing adjuvant medications (prednisone, dapsone, and others) and, later, IVIG to be withdrawn.…”