A Case of Bullous Pemphigoid: A Prevalent and Potentially Fatal Condition

Abstract

Bullous pemphigoid is the most common of the blistering disorders. It is most commonly found in the elderly and is diagnosed based on clinical, histologic, and immunologic criteria. It presents clinically with diffuse eczematous, pruritic, urticaria-like lesions, with the later appearance of tense bullae or blistering lesions typically filled with clear fluid. Histologically, a sub-epidermal blister is seen and immunofluorescence shows immunoglobulin G antibodies directed against the structural components of the keratinocytic hemidesmosomal proteins BP180 and BP230. Multiple treatment modalities are present for this condition, including anti-inflammatory medications, medications that reduce antibody formation, and treatments to increase the elimination of antibodies. The aim of this case report is to present a classic case of this condition, to highlight an awareness of differing treatment options, and to advocate referral to a dermatologist given its potential severity.

Doença Penfigóide bolhosa em lactentes - relato de caso

“…Na imunofluorescência indireta encontra-se IgG da subclasse IgG4, importante para o diagnóstico diferencial do penfigóide bolhoso em relação a outras dermatoses, como herpes gestacional, penfigóide cicatricial, epidermólise bolhosa adquirida, lúpus eritematoso sistêmico bolhoso, dermatite herpetiforme e dermatose por IgA linear. Desse modo, os níveis de anticorpos se relacionam diretamente com a atividade da doença 5,6 .…”

“…Outra classe de medicamentos consiste naqueles que diminuem a produção de anticorpos, como esteróides sistêmicos, azatioprina, metotrexato, micofenolato, ciclosporina e rituximabe. Finalmente, tratamentos que aumentam a eliminação de anticorpos anormais como plasmaferese e imunoglobulina intravenosa (IVIG) podem ser realizados 6 .…”

“…O PB localizado pode ser tratado com sucesso apenas com esteróides tópicos. Já a doença mais extensa, geralmente é tratada com agentes anti-inflamatórios e imunossupressores sistêmicos, como os corticosteróides orais 6 . As doses orais de prednisona/prednisolona variam de 0,3 a 1,25 mg/kg de peso corporal/dia, o que geralmente controla a doença em 1-2 semanas.…”

Bullous Pemphigoid: A Spontaneous Presentation in a Patient With Chronic Kidney Disease

“…The disease commonly occurs in the elderly population with more severity manifested in individuals over 50 years old with concurrent renal disease [ 1 - 3 ]. The dermatopathology highlights IgG antibodies that act against the structural aspects of keratinocytic hemidesmosomal proteins BP180 and BP230 [ 4 ]. In the United States, the overall prevalence of BP has been reported to be 0.012% per 100,000 individuals with incremental increases within each successive age group over the age of 60 years [ 5 ].…”

Bullous Pemphigoid- A Rare Case report

“…Anti-inflammatory drugs, pharmaceuticals that inhibit antibody production and treatments that enhance antibody elimination are among the therapy options available for this illness. The purpose of this case report is to describe a perfect illustration of this ailment, raise knowledge of different treatment choices, and urge for referral to a dermatologist due to its potential severity [1].…”