Incidence of Auto-Immune Pemphigus in the Midi-Pyrénées Region in 2002–2006

Abstract

Introduction: Auto-immune pemphigus is an organ-specific immune disorder due to pathogenic auto-antibodies. Both genetic and environmental factors have been associated with the occurrence of auto-immune pemphigus. Little is known about the epidemiology of auto-immune pemphigus in western Europe. Objective: To evaluate the incidence of auto-immune pemphigus in south-western France (namely the Midi-Pyrénées region) in a 5-year period between 2002 and 2006. Materials and Methods: We performed a retrospective study of the annual incidence of auto-immune pemphigus diagnosed in the Midi-Pyrénées region, between January 1, 2002, and December 31, 2006. Possible cases of pemphigus were initially selected using skin direct immunofluorescence (DIF) databases from all laboratories in the Midi-Pyrénées region. Systematic validation of all cases was performed by two of the authors (N.M., M.T.). To qualify as a case of auto-immune pemphigus, patients had to fulfil the following criteria: history of clinical signs of pemphigus as assessed by a dermatologist and a positive DIF. To be included in the study, validated cases of auto-immune pemphigus had to fulfil the following criteria: the date of first positive skin DIF between January 1, 2002, and December 31, 2006, and patient living in the Midi-Pyrénées region at the time of the first positive skin DIF. Results: Between 2002 and 2006, 91 patients with positive DIF were identified. Fifty-four patients with positive DIF did not meet the validation criteria for the study. Thirty-seven cases were included in the analysis. The crude annual mean incidence of auto-immune pemphigus between 2002 and 2006 was 2.7 cases/10⁶ inhabitants/year (95% CI: 1.87–3.69). The world-population-standardized annual mean incidence of auto-immune pemphigus was 1.55 (95% CI: 0.99–2.11). Conclusion: We report a 1.55/10⁶ inhabitants/year world-population-standardized incidence of auto-immune pemphigus in the south-west of France. A nationwide epidemiological study of pemphigus should be performed in France.

Afro‐Caribbean pemphigus: epidemiological data from a 5‐year prospective study on the island of Guadeloupe (French West Indies)

“…This first epidemiological study about autoimmune pemphigus in the Afro‐Caribbean population showed a high world‐population‐standardized incidence of 6.96 cases per million inhabitants per year (95% CI: 2.97–10.97). This incidence rate is higher than that reported in the USA and in Western Europe, where the incidence rate varies from 0.6 to 1.55 cases per million inhabitants per year according to the most recent studies . It is also greater than world‐population‐standardized incidence rates reported in studies performed in the Mediterranean Basin, where values are the highest (i.e.…”

“…Moreover, various epidemiological studies have identified environmental antigens linked to endemic PF . For these reasons, annual incidence of the disease differs among ethnic groups and in parts of the world . Little is known about the epidemiology of autoimmune pemphigus in black patients.…”

“…Little is known about the epidemiology of autoimmune pemphigus in black patients. Indeed, most studies concern Caucasians from Mediterranean Basin or Asia Minor and from Western Europe . Only two studies concerning black patients from South Africa and from Mali are available .…”

“…There are no epidemiological data regarding autoimmune pemphigus in the Afro‐Caribbean population. Moreover, all the studies performed with calculation of the world‐standardized incidence of autoimmune pemphigus are retrospective, and few include an individual validation of the cases in their study design …”

Epidemiological and clinical aspects of pemphigus in Senegal

“…Cette disparité serait probablement liée à des facteurs génétiques et environnementaux. En Occident, Asie et en Amérique latine, les particularités des pemphigus ont été bien caractérisées [3][4][5][6][7][8][9][10][11]. Par contre, en Afrique noire, l'étude des pemphigus a été souvent limitée par la Aspects epidemiologiques et cliniques des pemphigus Aspects epidemiologiques et cliniques des pemphigus au Senegal au Senegal non disponibilité des examens complémentaires, surtout des l'immunofluorescences, indispensables au diagnostic [12][13][14].…”