Bullous pemphigoid
An. bras. dermatol
; 94(2): 133-146, Mar.-Apr. 2019. tab, graf
Article
en En
| LILACS
| ID: biblio-1001150
Biblioteca responsable:
BR1.1
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
LILACS
Contexto en salud:
6_ODS3_enfermedades_notrasmisibles
Problema de salud:
6_skin_diseases
Asunto principal:
Penfigoide Ampolloso
Tipo de estudio:
Clinical_trials
/
Diagnostic_studies
/
Etiology_studies
Límite:
Aged
/
Humans
Idioma:
En
Revista:
An. bras. dermatol
Asunto de la revista:
DERMATOLOGIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Brasil