Pemphigus. Pemphigoid. Pemphigus familiaris benignus

Corticosteroid-Sparing Effect of Intravenous Immunoglobulin Therapy in Patients With Pemphigus Vulgaris

“…The lesions of PV can involve both cutaneous and mucosal tissues. [1][2][3] The diagnosis is confirmed by histological and immunopathological studies. [1][2][3][4][5] An intraepidermal vesicle with acantholysis is usually seen on routine histological examination of a biopsy specimen of the lesion.…”

“…[1][2][3] The diagnosis is confirmed by histological and immunopathological studies. [1][2][3][4][5] An intraepidermal vesicle with acantholysis is usually seen on routine histological examination of a biopsy specimen of the lesion. [1][2][3] Deposition of IgG on the surface of the keratinocyte cell surface is typically seen on direct immunofluorescence of perilesional tissue.…”

Novel Mechanisms of Target Cell Death and Survival and of Therapeutic Action of IVIg in Pemphigus

“…Hailey-Hailey disease) 57 that display cytopathological features characteristic of acantholytic cells of PV. 5 The morphology of acantholytic cells in PV does not match that of either typical apoptotic or oncotic cells. Because under certain circumstances, target cells have been reported to display features of both apoptosis and oncosis, such as a combination of an apoptotic nucleus and oncotic cytoplasm, termed the "apoptotic oncotic" cells, 58 we believe that simultaneous engagement of the apoptosis and oncosis effector mechanisms lead to cell death via acantholysis in PV.…”

“…50 Thereafter, tonofilaments retract into the perinuclear region, followed by disintegration of the desmosomes and the appearance of variable signs of degeneration, such as vacuolization, nuclear pyknosis, and abnormalities with the mitochondria and the endoplasmic reticulum. 5,51 In some mitochondria, cristae are preserved and are partially destroyed in the others. 52 After separation and eventual disappearance of desmosomes, residual cell membranes often show a conspicuous "villous" (bleb) formation.…”

“…4 PV patients represent a heterogeneous population with regard to the natural course of their disease, clinical features, and response to therapy, perhaps due to dramatic patient-to-patient variations in the immunopathological mechanisms. [5][6][7][8] Apoptosis is believed to play a role in the mechanism of keratinocyte death in PV. The occurrence of apoptosis markers has been observed in early lesions of PV patients before acantholysis.…”