Pemphigus
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Basics
Description
- Autoantibody (IgG)-mediated blistering disease of the skin and mucous membrane:
- Characterized by loss of cell-to-cell adhesion called acantholysis
- Median age 71 yr:
- Reports of disease occurring in neonates through elderly
- Rare; worldwide incidence 0.7/100,000
- Women more often than men (66%)
- Pemphix is Greek for bubble or blister
- Pemphigus – specific term for autoantibody disease–targeting desmogleins (Dsgs) which are cell-to-cell adhesion molecules
- Mortality is highest in those with mucocutaneous involvement:
- If untreated, mortality rates average 60–90%
- With treatment this nears 5%
- 3 major subtypes exist:
- Vulgaris; typically more serious with deeper mucocutaneous involvement:
- Accounts for 70–80% of all pemphigus
- Up to 70% with vulgaris present with oral lesions
- May or may not have skin involvement
- Flaccid bullae
- Autoantibodies to Dsg 1 and 3
- Affects most races in middle age and elderly Ashkenazi Jews
- Foliaceus; milder and more superficial cutaneous lesions:
- Involves skin only
- Autoantibodies to Dsg 1 only
- Paraneoplastic pemphigus; often with severe mucocutaneous involvement
- Most commonly seen in lymphoreticular malignancies
- Pemphigoid: A term describing the group of syndromes that cause a separation of the epidermis from the dermis, typically more benign course
- Bullous pemphigoid has tense bullae that arise from skin and are not as fragile as pemphigus
- Negative Nikolsky sign
- Often has pruritus prior to blistering (may be present for months)
- Oral lesions are rare
- Vulgaris; typically more serious with deeper mucocutaneous involvement:
Pediatric Considerations
- Pemphigus is rare in neonates and children but may occur in adolescents
- Early diagnosis and treatment significantly impact growth, psychological, social, and cultural development
- Histopathology is identical to adult disease
- Neonates may develop the disease secondary to transplacental transfer of IgG
- Neonatal pemphigus spontaneously resolves in several weeks as the maternal antibodies are catabolized
Pregnancy Considerations
- Effective treatment of maternal disease prior to conception lowers the risk of neonatal transmission and gestational complications
- Pregnancy may precipitate or aggravate pemphigus
Etiology
- IgG autoantibodies are directed against desmosomal cadherins Dsg 1 and Dsg 3 found in all keratinocytes
- Autoantibodies cause histopathologic acantholysis, cytoskeletal derangements, and apoptosis
- Bullae formation is caused by the loss of cell–cell adhesion and separation of the keratinocytes
- Immunogenetic predisposition secondary to higher frequencies of specific human leukocyte antigen HLA haplotypes including DR4 and DRw6
- Drugs such as penicillamine, captopril, rifampin, piroxicam, and phenobarbital can trigger pemphigoid reactions
- Endemic pemphigus foliaceus (fogo selvagem), most common in South America, may be triggered or transmitted by bites from flying insects
- Pemphigoid reactions may occur in association with a neoplasm, usually lymphoma (paraneoplastic pemphigus)
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
Description
- Autoantibody (IgG)-mediated blistering disease of the skin and mucous membrane:
- Characterized by loss of cell-to-cell adhesion called acantholysis
- Median age 71 yr:
- Reports of disease occurring in neonates through elderly
- Rare; worldwide incidence 0.7/100,000
- Women more often than men (66%)
- Pemphix is Greek for bubble or blister
- Pemphigus – specific term for autoantibody disease–targeting desmogleins (Dsgs) which are cell-to-cell adhesion molecules
- Mortality is highest in those with mucocutaneous involvement:
- If untreated, mortality rates average 60–90%
- With treatment this nears 5%
- 3 major subtypes exist:
- Vulgaris; typically more serious with deeper mucocutaneous involvement:
- Accounts for 70–80% of all pemphigus
- Up to 70% with vulgaris present with oral lesions
- May or may not have skin involvement
- Flaccid bullae
- Autoantibodies to Dsg 1 and 3
- Affects most races in middle age and elderly Ashkenazi Jews
- Foliaceus; milder and more superficial cutaneous lesions:
- Involves skin only
- Autoantibodies to Dsg 1 only
- Paraneoplastic pemphigus; often with severe mucocutaneous involvement
- Most commonly seen in lymphoreticular malignancies
- Pemphigoid: A term describing the group of syndromes that cause a separation of the epidermis from the dermis, typically more benign course
- Bullous pemphigoid has tense bullae that arise from skin and are not as fragile as pemphigus
- Negative Nikolsky sign
- Often has pruritus prior to blistering (may be present for months)
- Oral lesions are rare
- Vulgaris; typically more serious with deeper mucocutaneous involvement:
Pediatric Considerations
- Pemphigus is rare in neonates and children but may occur in adolescents
- Early diagnosis and treatment significantly impact growth, psychological, social, and cultural development
- Histopathology is identical to adult disease
- Neonates may develop the disease secondary to transplacental transfer of IgG
- Neonatal pemphigus spontaneously resolves in several weeks as the maternal antibodies are catabolized
Pregnancy Considerations
- Effective treatment of maternal disease prior to conception lowers the risk of neonatal transmission and gestational complications
- Pregnancy may precipitate or aggravate pemphigus
Etiology
- IgG autoantibodies are directed against desmosomal cadherins Dsg 1 and Dsg 3 found in all keratinocytes
- Autoantibodies cause histopathologic acantholysis, cytoskeletal derangements, and apoptosis
- Bullae formation is caused by the loss of cell–cell adhesion and separation of the keratinocytes
- Immunogenetic predisposition secondary to higher frequencies of specific human leukocyte antigen HLA haplotypes including DR4 and DRw6
- Drugs such as penicillamine, captopril, rifampin, piroxicam, and phenobarbital can trigger pemphigoid reactions
- Endemic pemphigus foliaceus (fogo selvagem), most common in South America, may be triggered or transmitted by bites from flying insects
- Pemphigoid reactions may occur in association with a neoplasm, usually lymphoma (paraneoplastic pemphigus)
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