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
An. bras. dermatol
;
85(4): 521-524, jul.-ago. 2010. ilus
Article
in Portuguese
| LILACS
| ID: lil-560583
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 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.
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Epidermolysis Bullosa Acquisita
/
Immunoglobulins, Intravenous
/
Immunologic Factors
Type of study:
Diagnostic study
Limits:
Female
/
Humans
Language:
Portuguese
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2010
Type:
Article
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