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Neonatal Autoimmune Blistering Disease: A Systematic Review
Cathy Y Zhao1,2, Yi Zhen Chiang1,2, Dedee F Murrell1,2
1Department of Dermatology, St. George Hospital, Sydney, New South Wales, Australia.
Pediatric Dermatology
|April 19, 2016
Summary
Neonatal autoimmune blistering diseases (AIBDs) can be passively transferred from mothers. Pemphigoid diseases present later and affect males more, while pemphigus appears at birth.
Area of Science:
- Dermatology
- Neonatology
- Immunology
Background:
- Neonatal autoimmune blistering diseases (AIBDs) are rare conditions.
- Understanding their pathogenesis, clinical features, and treatment is crucial for affected infants.
Purpose of the Study:
- To comprehensively review the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs).
- To differentiate characteristics between pemphigus and pemphigoid diseases in neonates.
Main Methods:
- Systematic literature search of Medline, Embase, PubMed, and LILACS from 1946 to December 2014.
- Inclusion of articles with confirmed AIBD diagnosis in infants under 4 weeks.
- Analysis of 51 identified cases, categorizing them into pemphigus and pemphigoid diseases.
Main Results:
- 51 cases identified: 34 pemphigus (31 PV, 3 PF) and 17 pemphigoid diseases (including BP, LABD).
- Pemphigoid diseases showed male predominance (4.6:1) and later onset (29.4% at birth) compared to pemphigus (1:1.06 ratio, 79.4% at birth).
- Trunk and head/neck were most affected; mucosal involvement occurred in 32.6%.
Conclusions:
- Maternal autoantibodies can cause AIBDs in newborns.
- Pemphigoid diseases are more common in males and tend to appear postnatally.
- Linear immunoglobulin A bullous dermatosis (LABD) may present differently from other AIBDs.
