Protection against Pemphigus Foliaceus by Desmoglein 3 in Neonates
Open Access
- 6 July 2000
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 343 (1) , 31-35
- https://doi.org/10.1056/nejm200007063430105
Abstract
Pemphigus foliaceus is an autoantibody-mediated blistering disease in which antibodies against desmoglein 1 cause loss of adhesion among keratinocytes in the superficial epidermis.1 Desmogleins are transmembrane glycoproteins found in desmosomes, which provide physical connections between cells. The main desmogleins expressed in the epidermis are desmoglein 1 and desmoglein 3. In adults, desmoglein 1 is present throughout the epidermis, but desmoglein 3 is present only in the basal and immediate suprabasal layers.2,3 It has been proposed that in patients with pemphigus foliaceus the antibodies interfere with the adhesive function of desmoglein 1 and that blisters occur only in the superficial epidermis, which contains desmoglein 1 without coexpressed desmoglein 3. In the unaffected deep epidermis, the presence of desmoglein 3 may compensate for the loss of function of desmoglein 1.2,3Keywords
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