ABSTRACT
Abstract: Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may be another cause of prerenal kidney failure. Further studies are needed to verify our hypothesis.
Subject(s)
Humans , Male , Aged , Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/pathology , Acute Kidney Injury/etiology , Skin/pathology , Biopsy , Epidermolysis Bullosa Acquisita/drug therapy , Treatment Outcome , Fluorescent Antibody Technique, Direct , Acute Kidney Injury/drug therapyABSTRACT
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.
Subject(s)
Female , Humans , Middle Aged , Epidermolysis Bullosa Acquisita/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Basement Membrane/pathology , /analysis , Epidermolysis Bullosa Acquisita/diagnosis , Epidermolysis Bullosa Acquisita/pathology , Fluorescent Antibody Technique, Direct , Immunoglobulin A/analysis , Immunoglobulin G/analysis , Severity of Illness Index , Treatment OutcomeABSTRACT
Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.
We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.
Subject(s)
Humans , Male , Middle Aged , Epidermolysis Bullosa Acquisita/pathology , Pemphigoid, Bullous/pathology , Skin/pathology , Anti-Inflammatory Agents/therapeutic use , Biopsy , Diagnosis, Differential , Epidermolysis Bullosa Acquisita/drug therapy , Pemphigoid, Bullous/drug therapy , Prednisone/therapeutic useABSTRACT
Os autores fazem uma revisäo da literatura sobre essa entidade e relatam 3 casos ocorridos no HU-UFJF no período de 1992 e 1998 colocando em evidência as principais características clínicas encontradas.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Epidermolysis Bullosa/pathology , Brazil , Dermatitis Herpetiformis/pathology , Epidermolysis Bullosa Acquisita/pathology , Pemphigoid, Bullous/pathology , Pemphigus/pathology , Porphyria Cutanea Tarda/pathology , Retrospective StudiesABSTRACT
Diversas enfermedades cutáneas se caracterizan por alteraciones localizadas preferentemente en la zona de membrana basal. Estas dermatosis se han hereditarias y adquiridas. Se pueden encontrar cambios ultraestructurales en la membrana basal, muchos de los cuales serían causados por fenómenos autoinmunes. Múltiples investigaciones han permitido aclarar la morfogénesis de las lesiones clínicamente visibles que afectan esta zona en particular. En la presente revisión se realiza una puesta al día de la última información de la literatura sobre estas interesantes enfermedades