RESUMO
A 74-year-old woman presented with a four-week history of locally expanding, highly pruritic urticarial plaques, bullae and erosions on her left breast. She had undergone surgery for an infiltrating ductal carcinoma of the same breast six months before and received intra- and postoperative radiotherapy (RT) followed by adjuvant hormonal anticancer treatment. Histopathological, immunological and serological data confirmed the diagnosis of localized bullous pemphigoid (BP) and treatment with systemic and local corticosteroids led to a sustained remission. After excluding other factors, we concluded that RT was the most likely trigger of her localized BP.
Assuntos
Doenças Mamárias/diagnóstico , Doenças Mamárias/etiologia , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/etiologia , Lesões por Radiação/diagnóstico , Lesões por Radiação/etiologia , Radioterapia Conformacional/efeitos adversos , Corticosteroides/uso terapêutico , Idoso , Anti-Inflamatórios/uso terapêutico , Doenças Mamárias/tratamento farmacológico , Feminino , Humanos , Penfigoide Bolhoso/tratamento farmacológico , Lesões por Radiação/tratamento farmacológicoRESUMO
Chronic involvement of orogenital and conjunctival mucosa in the course of either genetically based (epidermolysis bullosa hereditaria) or auto-immunologically mediated (as for example pemphigus vulgaris, mucous membrane pemphigoid or epidermolysis bullosa acquisita) blistering diseases can cause significant morbidity. To provide accurate care, recognition of clinical, pathogenic and diagnostic features as well as awareness of recent advances in the development of new therapeutic modalities are mandatory and thus will be discussed in this review.
Assuntos
Doenças Autoimunes/terapia , Vesícula/terapia , Doenças da Túnica Conjuntiva/terapia , Doenças dos Genitais Femininos/terapia , Doenças dos Genitais Masculinos/terapia , Doenças da Boca/terapia , Doenças Autoimunes/diagnóstico , Vesícula/diagnóstico , Feminino , Doenças dos Genitais Femininos/diagnóstico , Doenças dos Genitais Masculinos/diagnóstico , Humanos , Masculino , Doenças da Boca/diagnósticoRESUMO
Hereditary epidermolysis bullosa (EB) is a term for a heterogeneous group of rare genetic disorders characterized by marked fragility of the skin and mucous membranes following minor trauma. Significant progress has been made in understanding the molecular basis of EB, which has far-reaching implications for an improved classification with consequences for prognosis, genetic counseling, DNA-based prenatal and preimplantation testing, and the development of future treatments including gene therapy. Besides mucocutaneous changes, EB leads to a number of systemic manifestations whose management requires multidisciplinary access. Extracutaneous complications include ophthalmologic, dental, gastrointestinal, pulmonary, urogenital, hematologic, and nutritional problems. This article reviews the progress that has been made in the understanding of the molecular basis of EB, clinical aspects of major EB subtypes, and the management of patients suffering from EB, and it gives an outlook on molecular therapy projects such as gene, cell, vector, and protein therapies.