Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases
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 report...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Case Reports in Dermatological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2016/1653507 |
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| author | Ali Haydar Eskiocak Birgul Ozkesici Soner Uzun |
| author_facet | Ali Haydar Eskiocak Birgul Ozkesici Soner Uzun |
| author_sort | Ali Haydar Eskiocak |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-5f10ce6f311e4f86b52e008a19ad023c |
| institution | Kabale University |
| issn | 2090-6463 2090-6471 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Dermatological Medicine |
| spelling | doaj-art-5f10ce6f311e4f86b52e008a19ad023c2025-02-03T01:07:05ZengWileyCase Reports in Dermatological Medicine2090-64632090-64712016-01-01201610.1155/2016/16535071653507Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed CasesAli Haydar Eskiocak0Birgul Ozkesici1Soner Uzun2Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059 Antalya, TurkeyDepartment of Dermatology and Venereology, Akdeniz University School of Medicine, 07059 Antalya, TurkeyDepartment of Dermatology and Venereology, Akdeniz University School of Medicine, 07059 Antalya, TurkeyPemphigus 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.http://dx.doi.org/10.1155/2016/1653507 |
| spellingShingle | Ali Haydar Eskiocak Birgul Ozkesici Soner Uzun Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases Case Reports in Dermatological Medicine |
| title | Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases |
| title_full | Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases |
| title_fullStr | Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases |
| title_full_unstemmed | Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases |
| title_short | Familial Pemphigus Vulgaris Occured in a Father and Son as the First Confirmed Cases |
| title_sort | familial pemphigus vulgaris occured in a father and son as the first confirmed cases |
| url | http://dx.doi.org/10.1155/2016/1653507 |
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