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Pathology - Bullous pemphigoid
It is an immunobullous dermatological condition characterized by the presence of autoantibodies targeting epidermal hemidesmosomal proteins.
Epidemiology • The most prevalent immunobullous skin disorder, however still an uncommon condition with an annual incidence of 7 per million individuals. • The majority of cases occur in older adults over the age of 70. Aetiology • Generation of autoantibodies targeting epidermal hemidesmosomal proteins. • The principal antigens are identified as BPAg1 and BPAg2.
Pathogenesis • Antibody binding results in complement fixation and the influx of inflammatory cells, notably eosinophils. • Direct cytotoxic effects disrupt the hemidesmosomes that anchor the epidermis to the dermis, causing complete separation of the epidermis from the dermis.
Presentation • Characteristic skin lesions consist of substantial, tight, intact blisters that arise on either normal or erythematous skin. • Preferred locations include the lower trunk, inner thighs, forearms, axillae, and groin regions.
Histopathology • Biopsies reveal a subepidermal blister populated by numerous eosinophils. • The underlying dermis exhibits edema and is infiltrated by a significant inflammatory response rich in eosinophils. Direct immunofluorescence on perilesional skin demonstrates linear deposition of IgG and C3 along the basement membrane zone.
Prognosis • Mortality rates remain low with suitable immunosuppressive protocols. • The majority of problems are associated with therapy.
It is an immunobullous dermatological condition characterized by the presence of autoantibodies targeting epidermal hemidesmosomal proteins.
Epidemiology • The most prevalent immunobullous skin disorder, however still an uncommon condition with an annual incidence of 7 per million individuals. • The majority of cases occur in older adults over the age of 70. Aetiology • Generation of autoantibodies targeting epidermal hemidesmosomal proteins. • The principal antigens are identified as BPAg1 and BPAg2.
Pathogenesis • Antibody binding results in complement fixation and the influx of inflammatory cells, notably eosinophils. • Direct cytotoxic effects disrupt the hemidesmosomes that anchor the epidermis to the dermis, causing complete separation of the epidermis from the dermis.
Presentation • Characteristic skin lesions consist of substantial, tight, intact blisters that arise on either normal or erythematous skin. • Preferred locations include the lower trunk, inner thighs, forearms, axillae, and groin regions.
Histopathology • Biopsies reveal a subepidermal blister populated by numerous eosinophils. • The underlying dermis exhibits edema and is infiltrated by a significant inflammatory response rich in eosinophils. Direct immunofluorescence on perilesional skin demonstrates linear deposition of IgG and C3 along the basement membrane zone.
Prognosis • Mortality rates remain low with suitable immunosuppressive protocols. • The majority of problems are associated with therapy.
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