The most frequent subtypes of autoimmune pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). They are caused mainly by antibodies against desmoglein (Dsg) 1 and Dsg3. Sera from patients with mucocutaneous PV contain both anti-Dsg1 and anti-Dsg3 autoantibodies, whereas sera from patients with PF have anti-Dsg1 autoantibodies only. The transition from PV to PF has been rarely described in the literature and the mechanism that leads to a shift in autoantibodies profile remains unknown. We report a case of long-standing recalcitrant PV, that showed a transition to PF after the administration of etanercept 50 mg in order to threat toxic epidermal necrolysis (TEN) arisen after rituximab administration.
Transition from Pemphigus Vulgaris to Pemphigus Foliaceus after Toxic Epidermal Necrolysis / Di Zenzo, Giovanni; Didona, Dario; Paolino, Giovanni; Didona, Biagio. - (2016). (Intervento presentato al convegno 13th European Academy of Dermatology EADV Spring Symposium tenutosi a Athens, Greece).
Transition from Pemphigus Vulgaris to Pemphigus Foliaceus after Toxic Epidermal Necrolysis
Giovanni Paolino;
2016
Abstract
The most frequent subtypes of autoimmune pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). They are caused mainly by antibodies against desmoglein (Dsg) 1 and Dsg3. Sera from patients with mucocutaneous PV contain both anti-Dsg1 and anti-Dsg3 autoantibodies, whereas sera from patients with PF have anti-Dsg1 autoantibodies only. The transition from PV to PF has been rarely described in the literature and the mechanism that leads to a shift in autoantibodies profile remains unknown. We report a case of long-standing recalcitrant PV, that showed a transition to PF after the administration of etanercept 50 mg in order to threat toxic epidermal necrolysis (TEN) arisen after rituximab administration.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
