2000•New England Journal of MedicineOpen access

Protection against Pemphigus Foliaceus by Desmoglein 3 in Neonates

Hong Gyun Wu, Zhi Hong Wang, Albert C. Yan, Stephen R. Lyle, Steven S. Fakharzadeh, James K. Wahl, Margaret J. Wheelock, Hiroyasu Ishikawa, Jouni Uitto, Masayuki Amagai, John R. Stanley

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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 . . .

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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 . . .

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Available 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 . . .

Key concepts: Pemphigus foliaceus, Desmoglein 1, Desmoglein, Desmosome, Desmoglein 3, Staphylococcal scalded skin syndrome, Medicine, Pemphigus

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