ABSTRACT
El penfigoide gestacional es una dermatosis rara, que se presenta durante el embarazo. Se caracteriza por una respuesta autoinmune contra las proteínas de los hemidesmosomas, que genera un clivaje entre la epidermis y la dermis tanto de la piel como de las mucosas. Clínicamente, presenta prurito intenso, placas y pápulas eritematosas, que evolucionan a apollas con distribución en el abdomen y los miembros. Como complicaciones, en el feto puede generar parto prematuro y bajo peso para la edad gestacional, con alto riesgo de mortalidad. (AU)
Gestational pemphygoid is a rare, autoimmune dermatosis that occurs during pregnancy. It is characterized by an autoimmune response against hemidesmosome proteins, generating a cleavage between the epidermis and the dermis in the skin and mucous membranes. Clinically it presents with intense pruritus, plaques and erythematous papules that evolve to blisters that are distributed mainly in the abdomen and limbs. The complications are preterm birth and low weight for gestational age, with high risk of mortality. (AU)
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Pemphigoid Gestationis/diagnosis , Pemphigoid, Bullous/diagnosis , Prednisone/analogs & derivatives , Pemphigoid Gestationis/drug therapy , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/drug therapy , Diabetes, Gestational/diagnosis , Glucocorticoids/therapeutic useABSTRACT
Abstract: Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
Subject(s)
Humans , Aged , Pemphigoid, Bullous/diagnosis , Steroids/therapeutic use , Autoimmunity/physiology , Fluorescent Antibody Technique/methods , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/drug therapy , Diagnosis, DifferentialABSTRACT
El pengigoide (PMB o penfigoide de las membranas mucosas PMM) es una enfermedad de las mucosas, piel o de piel y mucosas, de naturaleza autoinmune que se caracteriza por la formación de ampollas. En esta ocasión se presenta una revisión de 55 casos, realizada en el IREPO (Instituto de Referencia de Patología Oral) y en el Servicio de diagnósticos de la Escuela Dental, entre los años 1978 y 1998, en todos los pacientes el diagnóstico fue confirmado con biopsia
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Gingival Diseases/diagnosis , Palate, Soft , Pemphigoid, Bullous/diagnosis , Adrenal Cortex Hormones/therapeutic use , Betamethasone/therapeutic use , Biopsy , Clinical Evolution , Hydroxychloroquine/therapeutic use , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/etiologyABSTRACT
Penfigóide bolhoso (PB) é raro na criança e no adulto jovem. Relatamos aqui o caso de um paciente de 22 anos que apresentou erupçäo bolhosa com características clínicas e imunopatológicas de PB induzido por medicaçäo antigripal (ácido acetil-salicílico + cafeína). Näo existem relatos na literatura de PB provocado por essa associaçäo medicamentosa. Säo feitas consideraçöes sobre a participaçäo de drogas no desencadeamento de PB possíveis associaçöes com doenças auto-imunes