Clinical and Immunologic Characterization in 26 Indian Pemphigus Patients

Abstract

Background: Pemphigus shows geographically variable characteristics.Objective: To study the clinical and immunologic characteristics of Indian pemphigus patients before and after treatment. Methods: Twenty-six Indian pemphigus patients were analyzed with regard to age, gender, clinical features, treatments and response, the results of histopathology, direct and indirect immunofluorescence (IF), enzyme-linked immunosorbent assay (ELISA), and immunoblot analyses.Results: There were 22 pemphigus vulgaris (PV) and 4 pemphigus foliaceus (PF) patients. Direct and indirect IF was positive in 95.8% and 56% of patients, respectively. Indices of ELISA were lower in our study. Immunoblot assays detected the 130 kDa desmoglein-3 in 10 PV patients and the 160 kDa desmoglein-1 in 1 PV patient; 190 kDa periplakin was unexpectedly detected in 8 patients.Conclusion: Indian pemphigus patients showed several unique characteristics, including younger population, predominance of PV, low ELISA indices, lower sensitivity of indirect IF and immunoblotting, and the presence of the 190 kDa periplakin in nearly onethird of patients.Contexte: Le pemphigus pré sente des caracté ristiques diffé rentes selon les ré gions. Objectif: L'é tude visait à dé terminer les caracté ristiques cliniques et immunologiques du pemphigus chez des patients indiens, avant et aprè s le traitement. Mé thode: Divers facteurs, soit l'â ge, le sexe, les manifestations cliniques, le traitement, et la ré action au traitement ainsi que les ré sultats de l'examen histopathologique, de l'immunofluorescence (IF) directe et indirecte, du titrage immunoenzymatique (mé thode ELISA), et de l'immunobuvardage, ont fait l'objet d'analyse chez 26 patients indiens atteints de pemphigus. Ré sultats: Ont é té relevé s 22 cas de pemphigus vulgaire (PV) et 4 cas de pemphigus foliacé (PF). L'IF directe et indirecte s'est ré vé lé e positive chez 95.8% et 56% des patients, respectivement. Les titrages selon la mé thode ELISA é taient faibles dans cette é tude.Quant à l'immunobuvardage, il a permis de dé tecter la pré sence de desmoglé ine-3 de 130 kDa chez 10 patients atteints de PV et de desmoglé ine-1 de 160 kDa chez 1 patient atteint de PV, mais, dé couverte surprenante, la pré sence de pé riplakine de 190 kDa a é té dé celé e chez 8 patients.Conclusions: Plusieurs caracté ristiques particuliè res ont é té relevé es chez les Indiens atteints de pemphigus, notamment l'â ge peu avancé , la pré dominance du PV, de faibles titrages selon la mé thode ELISA, une faible sensibilité de l'IF indirecte et de l'immunobuvardage, et la pré sence de pé riplakine de 190 kDa chez presque un tiers des patients.

Clinical and immunological study of mucous membrane pemphigoid in a cohort of Indian patients

“…Antibodies directed against the 190‐kDa periplakin were found in two patients. Similar findings were found in our previous study on Indian pemphigus patients . None of these patients showed antibodies to both periplakin and envoplakin, the hallmark of paraneoplastic pemphigus.…”

A misleading procedure name may prove dangerous: cautionary note on an article by Har‐Shai et al.

“…Similar findings were found in our previous study on Indian pemphigus patients. 6 None of these patients showed antibodies to both periplakin and envoplakin, the hallmark of paraneoplastic pemphigus.…”

Summary of results of serological tests and diagnoses for 4774 cases of various autoimmune bullous diseases consulted to Kurume University

“…All sera were kept at −80 °C for long durations and at 4 °C with 0·1% sodium azide as a preservative during experiments. Studies of the considerably large number of patients used in this study have previously been reported . All previously reported studies for smaller numbers of patients used in this study are listed in Appendix S2 (see Supporting Information).…”