Pemphigus vulgaris in siblings: HLA-DR4 and HLA-DQw3 and susceptibility to pemphigus.

Abstract

BACKGROUND:

The association of pemphigus vulgaris with the HLA serotypes, DR4 and DRw6, and with the DQ-beta chain alleles, DQw1 and DQw3, suggests that there is a genetic predisposition to this disease. However, familial cases of pemphigus vulgaris are exceedingly rare.

OBJECTIVE:

We studied two siblings with pemphigus vulgaris and three unaffected family members to determine whether an HLA allele was associated with the development of pemphigus vulgaris in this family.

METHODS:

We utilized restriction fragment length polymorphism methods using the HLA-DR beta 1, HLA-DQ alpha, and HLA-DW beta 1 genes as cDNA probes.

RESULTS:

We found that the affected siblings share the haplotype HLA-DR4 and the DQw.3.2 allele.

CONCLUSION:

Our findings of gene sharing among siblings with pemphigus vulgaris lend support to previous studies of unrelated Caucasian patients, which implicated DQw3 allele polymorphisms in conferring susceptibility to pemphigus.

• Authors: P B Reohr, A Mangklabruks, A M Janiga, L J DeGroot, Y Benjasuratwong, K Soltani
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 27 Issue 2 Pt 1 Pg. 189-93 (Aug 1992) ISSN: 0190-9622 [Print] United States
• PMID: 1358926 (Publication Type: Case Reports, Journal Article)

Chemical References

• DNA Probes
• HLA-DQ Antigens
• HLA-DR4 Antigen
• HLA-DR6 Antigen

Topics

• Adult
• Alleles
• DNA Probes
• Female
• HLA-DQ Antigens (analysis)
• HLA-DR4 Antigen (analysis)
• HLA-DR6 Antigen (analysis)
• Humans
• Pemphigus (genetics, immunology)
• Polymorphism, Restriction Fragment Length