- Published on
Pathology - Pemphigus vulgaris
Definition: An immunobullous dermatosis resulting from autoantibodies targeting epidermal desmosomal proteins.
Epidemiology • Infrequent, occurring at a rate of 0.1–1 per 100,000 individuals annually. • Typically impacts middle-aged persons aged 40–60 years.
Aetiology: Production of autoantibodies targeting the epidermal desmosomal cadherin, desmoglein-3.
Pathogenesis • The autoantibody attaches to the extracellular domain of desmoglein-3, resulting in desmosomal impairment and acantholysis. • The conventional perspective posited that complement fixation resulted in acantholysis; however, other researchers have proposed that acantholysis may arise via cytoskeletal collapse independent of complement activity.
Exposition • The majority of cases commence with oral erosions and blisters, then followed weeks or months later by the emergence of cutaneous lesions. The cutaneous lesions are delicate vesicles arising on unremarkable or erythematous skin. The blisters readily burst, resulting in a painful eroded region. • The skin lesions generally manifest on the face, scalp, axillae, and groin.
Histopathology • Biopsies reveal a blister cavity within the epidermis populated by acantholytic keratinocytes. • Generally, the cleavage occurs at a suprabasal level, resulting in the blister floor being lined by a solitary layer of intact basal keratinocytes. • Acantholysis may also affect the epidermis of adnexal structures. • Typically, there exists a fundamental dermal inflammatory infiltrate characterized by a significant presence of eosinophils.
Immunofluorescence
Direct immunofluorescence on perilesional skin demonstrates the deposition of IgG and C3 in the intercellular space of the epidermis.
Prognosis: Mortality rates are minimal with suitable immunosuppressive therapies. • The majority of problems are associated with therapy.
Definition: An immunobullous dermatosis resulting from autoantibodies targeting epidermal desmosomal proteins.
Epidemiology • Infrequent, occurring at a rate of 0.1–1 per 100,000 individuals annually. • Typically impacts middle-aged persons aged 40–60 years.
Aetiology: Production of autoantibodies targeting the epidermal desmosomal cadherin, desmoglein-3.
Pathogenesis • The autoantibody attaches to the extracellular domain of desmoglein-3, resulting in desmosomal impairment and acantholysis. • The conventional perspective posited that complement fixation resulted in acantholysis; however, other researchers have proposed that acantholysis may arise via cytoskeletal collapse independent of complement activity.
Exposition • The majority of cases commence with oral erosions and blisters, then followed weeks or months later by the emergence of cutaneous lesions. The cutaneous lesions are delicate vesicles arising on unremarkable or erythematous skin. The blisters readily burst, resulting in a painful eroded region. • The skin lesions generally manifest on the face, scalp, axillae, and groin.
Histopathology • Biopsies reveal a blister cavity within the epidermis populated by acantholytic keratinocytes. • Generally, the cleavage occurs at a suprabasal level, resulting in the blister floor being lined by a solitary layer of intact basal keratinocytes. • Acantholysis may also affect the epidermis of adnexal structures. • Typically, there exists a fundamental dermal inflammatory infiltrate characterized by a significant presence of eosinophils.
Immunofluorescence
Direct immunofluorescence on perilesional skin demonstrates the deposition of IgG and C3 in the intercellular space of the epidermis.
Prognosis: Mortality rates are minimal with suitable immunosuppressive therapies. • The majority of problems are associated with therapy.
0 Comments
