RESUMO
@#Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone. The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological findings. The management depends on the clinical extent and severity. We present in this article a literature review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the Philippine Dermatological Society in the management of BP.
Assuntos
Penfigoide BolhosoRESUMO
@#<p style="text-align: justify;">Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.</p>