The epidermis and blistering disorders: pemphigus

Published on May 4, 2014   57 min
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The epidermis and blistering disorders. This lecture is on pemphigus.
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Pemphigus vulgaris usually presents on oral and genital mucosa, and may also involve skin. The lesions are fragile bullae that lead to shallow erosions that heal very slowly. Biopsy shows acantholytic suprabasal cleft. Direct immunofluorescence of biopsy reveals epidermal deposits of IgG and complement in the intercerllular space. Indirect immunofluorescence of serum demonstrates so-called "intercellular" antibodies reacting with the cell membrane of stratified squamous epithelium.
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Prior to the introduction of an effective therapy with oral coritcosteroids in the 1950s, the disease had a dismal natural course, with approximately 50% mortality rate at two years and about 100% mortality rate by five years after the onset of the disease. The mortality rate now is estimated at about 5%, and death is almost invariably due to complications of immunosuppressive therapy.
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Prognosis of pemphigus. Complete and long-lasting remissions, such as no evidence of the disease and no systemic therapy required for at least six months, can occur in about 25%, 50%, and 75% of patients 2, 5, and 10 years after diagnosis, respectively. Patients with mild or moderate disease and diagnosis are twice as likely to enter a long-lasting, complete remission than those with severe disease. Patients who respond rapidly to treatment are over twice as likely to enter a long-lasting, complete remission than those with a slower response. If not promptly and aggressively treated, pemphigus hardens, providing the opportunity for the spreading of epitopes and rendering the disease significantly more difficult to control.
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The epidermis and blistering disorders: pemphigus

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