Abstract Number: 24

Neonatal autoimmune blistering diseases -a systematic review of literature

C.Y. Zhao, N.Y.Z. Chiang, D.F. Murrell

Meeting: 2015 Dermcoll

Session Information

Date: -

Session Title: Paediatric Dermatology Symposium

Session Time: -

Introduction: Neonatal autoimmune blistering disease
(AIBD) is rare. To the best of our knowledge, this is the first
systematic review of neonatal AIBD, aimed at fostering a
better understanding of neonatal AIBD’s clinical presentation
and prognosis.
Method: The literature was searched using MEDLINE,
Embase, PubMed, LILACS and reference lists of identified
papers. Inclusion criteria were: all time periods and
languages; neonates were defined as <4 weeks old; has a confirmed diagnosis of AIBD (positive skin signs, histopathology and autoantibodies). Cases were analysed overall and by subtypes. Results: We reviewed 425 abstracts and 100 full-text papers to identify 44 cases of neonatal AIBD in 42 papers, including 2 pairs of twins. • Subtypes: 29(65.9%) pemphigus vulgaris, 3(6.8%) pemphigus foliaceus, 4(9.1%) bullous pemphigoid(BP), 5(11.4%) linear IgA dermatosis(LABD), 1(2.3%) epidermolysis bullosa acquisita(EBA), 1(2.3%) concomitant BP&LABD and 1(2.3%) bullous lupus. • Sex distribution(42 cases with data): 25 males(59.5%),17 females(40.5%) • Body site involvement (39 cases with data): head and neck(in 52.5% of cases) and trunk(52.5%) were the commonest sites, followed by extremities(43.6%), legs(41.0%), mucosa(35.9%), arms(15.4%) and genitals(10.3%). • Onset: 29(65.6%) presented at birth, 6(13.6%) presented on day 1, and the rest presented on average on day 6. Pemphigus more commonly presents at birth(26 of 32, 81.3%) compared to pemphigoids(3 of 11, 18.2%). • Outcome: 3(6.8%) achieved remission within a week, 32(72.7%) achieved remission within a month, 3(6.8%) achieved remission within 6 months, 2(4.5%) required ongoing maintenance therapy, 3(6.8%) stillborns and 1(2.3%) died on 10 days after birth. By subtype, LABD has the worst prognosis, often with airway complications and slow remission.