Oral pemphigoid. Subset of cicatricial pemphigoid?

OBJECTIVE: Cicatricial pemphigoid (CP) is an autoimmune blistering disease characterized by anti-basement membrane zone (BMZ) antibodies with a varied heterogeneous clinical spectrum. We sought to characterize a subset of patients with disease limited to the oral cavity. STUDY DESIGN: Twenty-nine random patients with vesiculobullous disease limited to the oral cavity were studied. We identified patients by clinical criteria, the presence of subepidermal/subepithelial bullae on routine histopathologic study, and deposition of IgG, complement, or both on the BMZ of perilesional tissue by immunopathological studies. Treatment included local and systemic therapies. Patients were monitored for a mean period of 6.7 years (range, 3.5 to 11 years). RESULTS: The female/male ratio was 4.8:1. Patients with limited or minimal disease received local therapy only. In patients with extensive or severe disease, the use of dapsone yielded significant clinical improvement. Long-term follow-up showed that patients with severe disease treated with dapsone followed a clinical course similar to that in patients with minimal disease. CONCLUSIONS: Oral pemphigoid is a distinct clinical subset of CP. Overall it has a relatively benign course compared with that in patients with CP involving the oral cavity and other mucosae and the skin. Patients with minimal disease respond satisfactorily to topical therapy. Patients with severe and extensive disease benefit from dapsone therapy. In most patients the clinical course is prolonged and treatment is required for several months; in our study the mean treatment period was 42 months (range, 24 to 78 months). All the patients in this study went into clinical remission and remained in remission on cessation of therapy. No other mucosae or the skin were involved during the follow-up period.

Identical MHC markers in non-Jewish Iranian and Ashkenazi Jewish patients with pemphigus vulgaris: possible common central Asian ancestral origin.

Previous studies showed that almost all Ashkenazi Jewish patients with pemphigus vulgaris carried the extended haplotype [HLA-B38, SC21, DRB1*0402, DQB1*0302] or [HLA-B35, SC31, DRB1*0402, DQB1*0302] or class II fragments of them. Non-Jewish patients carried [HLA-B55, SB45, DRB1*1401, DQB1*0503] or its class II fragments. In the present study of 20 Iranian patients with pemphigus vulgaris, 17 were found to carry DRB1*0402, DQB1*0302 haplotypes, also found among normal Iranian haplotypes and the same as that of the Jews. These findings suggest that the pemphigus MHC susceptibility gene among Iranians derived from the same ancestor as that in the Ashkenazim. The ancient Jews were under Persian domination from 500 B.C. until 300 B.C. and in the 8th century A.D., a Tataric people living in the kingdom of Khazar on the Western shore of the Caspian Sea and the Northern shore of the Black Sea, near Persia, converted to Judaism, providing possible opportunities for gene mixing in two populations that are distinct and separate today.

Intravenous immunoglobulins in the therapy of autoimmune and systemic inflammatory disorders.

OBJECTIVE: The focus of this review is to summarize the mechanism and the adverse side-effects of Intravenous Immunoglobulin (IVIg) therapy, and to highlight the current cumulative experience of its use in the treatment and management of autoimmune and systemic inflammatory diseases. DATA SOURCES: Detailed search of the literature was done. Studies involving only humans were considered for clinical evaluation. Animal studies were used only for understanding mechanisms of action. The NIH Consensus Conference of May 1990 and the Australian Society for Blood Transfusion of July 1993 were used for guidelines. STUDY SELECTION: Material was taken only from peer reviewed journals. RESULTS: It appears that IVIg may act by more than one mechanism of action. It is unclear whether the mechanism is different in different diseases and whether more than one mechanism may apply to any disease or clinical state. The incidence and gravity of serious side effects appears low. It is the mainstay of treatment of immune thrombocytopenia purpura and Kawasaki disease. CONCLUSION: IVIg is a safe and effective therapeutic modality that can be added to the repertoire of various agents used to treat autoimmune and systemic inflammatory diseases. Long-term prospective studies are needed to define indications, dose-schedules, duration of therapy, and influence on the clinical courses of chronic diseases better.

Common major histocompatibility complex class II markers in clinical variants of cicatricial pemphigoid.

Cicatricial pemphigoid (CP) is a chronic autoimmune blistering disease affecting multiple mucous membranes derived from stratified squamous epithelium and occasionally the skin. CP has a wide spectrum of disease manifestations. Patients with oral pemphigoid (OP) have a benign self-limited disease in which pathological changes are restricted to the oral mucosa. On the other hand, patients with ocular cicatricial pemphigoid (OCP), a chronic condition marked with relapses and remissions, have ocular involvement and also perhaps involvement of other mucous membranes. All clinical subsets are characterized by the presence of a similar anti-basement zone autoantibody. The factors that determine the development of one form of CP or the other are not known. In a previous study, we described the association between OCP and the DQB1*0301 allele (P = 0.006). In this study, we have analyzed 22 Caucasian patients with OP and their family members for major histocompatibility complex DRB generic, DQA1, and DQB1 allele associations by PCR-sequence-specific oligonucleotide probe hybridization. The results were compared to those obtained from 17 Caucasian patients with OCP and to control Caucasian alleles and haplotypes. The DQB1*0301 allele frequency was 38.6% in OP, 52.9% in OCP, and 17.8% in controls. Statistically significant associations were detected between the DQB1*0301 allele and both OP (P = 0.0047) and OCP (P < 0.0001). In addition, DRB1*04 showed a statistically significant association (P = 0.005) with OCP when compared to controls. Analysis of major histocompatibility complex class II haplotypes showed significant statistical associations between both OCP and OP and the HLA-DRB1*04, DRB4*0101, DQA1*03, DQB1*0301 haplotype (P < 0.0001 and P = 0.0012, respectively). Our results indicate that DQB1*0301 is a marker of both oral and ocular forms of CP. The analysis of the amino acid sequence of the DQB1 alleles present in both OP and OCP suggested that amino acid residues at position 57 and positions 71-77 may also be markers of CP.