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CURRENT ISSUEGIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases

Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014

Frequency: Bi-Monthly

ISSN 0392-0488

Online ISSN 1827-1820

 

Giornale Italiano di Dermatologia e Venereologia 2007 December;142(6):715-8

    CASE REPORTS

Familial pemphigus vulgaris

Calista C. 1, Cerbone L. 2, Morri M. 3

1 Unit of Dermatology M. Bufalini Hospital, Cesena, Italy
2 Plinio Clinical Center Ercolano, Napoli, Italy
3 Department of Dermatology Ospedale per gli Infermi, Rimini, Italy

Pemphigus (P) is an autoimmune disease which occurs due to both a combination of environmental trigger factors and a complex genetic predisposition. The onset of P in a familial setting is very rare as only 31 families in which more than one family member was involved have been reported. On reviewing the literature, we found that in 17 of the 31 families P was diagnosed in siblings. In this group of patients, the average age at diagnosis was 46.7 years for the proband (the first subject affected), and 45.1 years for the sibling. In the remaining 14 families one of the parents and one of the children were affected. The median age at diagnosis for the first generation was 54.4 years, and 38.3 for the second. Evidence of anticipation, defined as a younger age at diagnosis in successive generations, was observed in this group of patients, with 75% of cases showing an anticipation of over 20 years. Considering the low incidence of familial P in the general population, the likelihood of inherited genetic factors being responsible for P currently appears difficult to prove. Members belonging to families with P provide us with a unique opportunity to understand the etiopathogenetic aspects of this autoimmune disease. We report a family in which mother and son were affected by P.

language: English


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