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Case Reports in Dermatological Medicine
Volume 2016, Article ID 1653507, 4 pages
http://dx.doi.org/10.1155/2016/1653507
Case Report

Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases

Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059 Antalya, Turkey

Received 26 December 2015; Revised 9 April 2016; Accepted 15 May 2016

Academic Editor: Alireza Firooz

Copyright © 2016 Ali Haydar Eskiocak et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease of the skin and mucous membranes. Although there is some evidence pointing towards a genetic predisposition by some human leukocyte antigen (HLA) genes, familial occurrence of PV is very rare. Most of the familial PV cases so far reported have been in mother and daughter and in siblings. PV in father and son, as presented here, has not been reported in the literature before, except an unconfirmed report. The diagnosis of PV was established by histologic, cytologic studies and enzyme linked immunosorbent assay (ELISA) in Case and by ELISA and BIOCHIP indirect immunofluorescence test in Case . The son was responsive to moderate doses of methylprednisolone, with the treatment continuing with tapered doses. The father was in a subclinic condition; consequently, only close follow-up was recommended. HLA typing studies revealed identical HLA alleles of HLA-DR4 () and HLA- in both of our cases; this had been found to be associated with PV in prior studies. Familial occurrences of PV and related HLA genes indicate the importance of genetic predisposition. The first occurrence of confirmed familial PV in father and son is reported here.