PMID- 27403352
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160712
LR  - 20201001
IS  - 2090-6463 (Print)
IS  - 2090-6471 (Electronic)
IS  - 2090-6463 (Linking)
VI  - 2016
DP  - 2016
TI  - Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed 
      Cases.
PG  - 1653507
LID - 10.1155/2016/1653507 [doi]
LID - 1653507
AB  - 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 1 and by ELISA and BIOCHIP indirect 
      immunofluorescence test in Case 2. 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 (DRB1(⁎)04) and 
      HLA-DQB1(⁎)03 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.
FAU - Eskiocak, Ali Haydar
AU  - Eskiocak AH
AD  - Department of Dermatology and Venereology, Akdeniz University School of Medicine, 
      07059 Antalya, Turkey.
FAU - Ozkesici, Birgul
AU  - Ozkesici B
AD  - Department of Dermatology and Venereology, Akdeniz University School of Medicine, 
      07059 Antalya, Turkey.
FAU - Uzun, Soner
AU  - Uzun S
AD  - Department of Dermatology and Venereology, Akdeniz University School of Medicine, 
      07059 Antalya, Turkey.
LA  - eng
PT  - Journal Article
DEP - 20160615
PL  - United States
TA  - Case Rep Dermatol Med
JT  - Case reports in dermatological medicine
JID - 101591808
PMC - PMC4925942
EDAT- 2016/07/13 06:00
MHDA- 2016/07/13 06:01
PMCR- 2016/06/15
CRDT- 2016/07/13 06:00
PHST- 2015/12/26 00:00 [received]
PHST- 2016/04/09 00:00 [revised]
PHST- 2016/05/15 00:00 [accepted]
PHST- 2016/07/13 06:00 [entrez]
PHST- 2016/07/13 06:00 [pubmed]
PHST- 2016/07/13 06:01 [medline]
PHST- 2016/06/15 00:00 [pmc-release]
AID - 10.1155/2016/1653507 [doi]
PST - ppublish
SO  - Case Rep Dermatol Med. 2016;2016:1653507. doi: 10.1155/2016/1653507. Epub 2016 
      Jun 15.