Dermatoses bolhosas auto-imunes

Abstract

Resumo: Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme. Palavras-chave: Alergia e imunologia; Dermatite herpetiforme; Dermatopatias vesiculobolhosas; Penfigo; Penfigóide bolhoso Abstract: Abstract: Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis. Keyword: Allergy and immunology; Bullous pemphigoid; Dermatitis herpetiformis; Pemphigus; Skin diseases vesiculobullous histológicas comuns e ambos apresentam autoanticorpos da subclasse IgG4, cujo antígeno-alvo é a desmogleína 1, antígeno desmossomal de 160kd.O FS acomete mais frequentemente crianças, adolescentes e adultos jovens que vivem nas áreas rurais de regiões endêmicas, e é comum a ocorrência

Penfigoide de membranas mucosas com estenose esofágica grave

“…It is currently defined as a heterogenous group of chronic subepidermal bullous diseases that predominantly affect the mucous membranes. 1 The condition is also known as cicatricial pemphigoid, benign mucous membrane pemphigoid, oral pemphigoid and ocular cicatricial pemphigoid. [1][2] Involvement of the esophagus in mucous membrane pemphigoid is rare and is usually found in patients with a disseminated form of the disease.…”

“…1 The condition is also known as cicatricial pemphigoid, benign mucous membrane pemphigoid, oral pemphigoid and ocular cicatricial pemphigoid. [1][2] Involvement of the esophagus in mucous membrane pemphigoid is rare and is usually found in patients with a disseminated form of the disease. The most common alterations are the involvement of multiple mucous membranes or esophageal strictures.…”

“…5 These autoantibodies are varied and are specific to various antigens such as bullous pemphigoid antigen 2, epilegrin and type VII collagen, among others. 1,6,7 As a function of the way in which each patient reacts to the autoantibodies involved, cicatricial pemphigoid may be classified into four different subgroups: the first group, in which patients present autoantibodies against laminin 5, cannot be distinguished clinically from the other groups; in the second group, the ocular mucosa is predominantly affected; in the third group, lesions are present in both the skin and the mucous membranes; while the fourth group encompasses patients in whom various mucous membranes may be affected but there is no skin involvement. In the present case, positivity was for IgG and the pattern was linear, which does not distinguish it from acquired epidermolysis bullosa or bullous pemphigoid.…”

“…In the second group, immunosuppressive therapy may include pulse methylprednisolone, cyclophosphamide, rituximab and even intravenous immunoglobulin in refractory cases. 2,[9][10][11][12][13][14] The patient in question, who was classified as high-risk because of her ocular and esophageal lesions, was submitted to pulse therapy with methylprednisolone and intravenous immunoglobulin. Complete remission was achieved following the second form of treatment.…”

Imunossupressores na Dermatologia

“…Em monoterapia: buloses (quando há restrição ao uso de corticosteroide) 48,49 , pioderma gangrenoso; 50 Combinada com outras drogas: vasculites, colagenoses.…”