ABSTRACT
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
Se revisa la correlación histopatológica e inmunofluorescencia directa (IFD) de piel en 127 casos con los diagnósticos de pénfigo, penfigoide ampollar y dermatitis herpetiforme en el servicio de dermatología del Hospital Universitario de Caracas, durante un período de 15 años. El 88,46 por ciento de pacientes con pénfigo tuvo IFD positiva; los casos de penfigoide ampollar y dermatitis herpetiforme el 77,7 por ciento y 81,25 por ciento. La correlación histopatológica e IFD fue de 85,48 por ciento, 67,85 por ciento y 86,48 por ciento para pénfigo, penfigoide ampollar y dermatitis herpetiforme respectivamente