Clinicopathological features and HLA tissue typing in pemphigoid gestationis patients in Kuwait.

Pemphigoid gestationis (PG) is a rare autoimmune disease of pregnancy. We report a series of 22 cases of PG in Kuwait. They constituted 18% of all the autoimmune bullous diseases registered in our centre over a span of 11 years. PG was observed to be the third most common bullous disease in our region. Ninety-five per cent of the patients were of Arab ethnicity. The clinical features observed in our patients were comparable to those reported elsewhere. Systemic steroids (prednisolone 20-60 mg daily) remained the mainstay of treatment to control the active disease and an optimal dose of 20 mg of prednisolone was maintained throughout the pregnancy and immediate postpartum period. We observed a favourable outcome of pregnancies complicated by PG without any associated maternal or foetal morbidity. Kuwaiti patients with PG were observed to have a predominance of HLA-DR3 and DQ2 antigens. No predominance of HLA-DR4 antigen was observed.

Class II major histocompatibility complex typing across the ethnic barrier in pemphigoid gestationis. A study in Mexicans.

BACKGROUND: Pemphigoid gestationis (PG), also called herpes gestationis, is a rare autoimmune disease of pregnancy or puerperium (estimated 1 out of 50,000 pregnancies among Caucasians). A previous series has demonstrated an association of PG with human leukocyte antigen (HLA)-DR3 or HLA-DR4 haplotypes. While these haplotypes are most commonly found in individuals of European ancestry, they have also been found in African-American patients affected with PG. PG has rarely been reported in other ethnic groups, and the HLA association in non-Europeans has not been examined. METHODS: We have characterized eight patients of Mexican ancestry who have PG by clinical, histologic, and immunofluorescence criteria. Class I and class II major histocompatibility complex (MHC) antigens were studied by standard microlymphocytotoxicity assays. Class II MHC antigens were further studied by polymerase chain reaction (PCR) amplification of HLA-DRB1, DQA, and DQB genes and allele-specific oligonucleotide hybridization. For comparison purposes, we used results obtained from a group of 100 ethnically matched healthy individuals. RESULTS: We found that all eight patients had the HLA-DR3/DR4 phenotype; all HLA-DR3 haplotypes were HLA-DRB1*0301, DQA1*0501, and DQB1*0201, whereas half of the HLA-DR4 haplotypes were from the DRB1*0401 subtype and the other half were DRB1 *0407. CONCLUSIONS: These results suggest that, in Mexicans, the genetic susceptibility for the development of PG is strongly influenced by the genetic admixture of Caucasian origin, and the role of class II MHC antigens in the pathophysiology of this disease is confirmed.