ABSTRACT
This case report presents a 13-year-old boy referred to the Department of Paediatric Dentistry, Women's and Children's Hospital, Adelaide, South Australia, Australia, with a 5-week history of severe oral ulcerations and significant weight loss of unknown origin. The diagnosis of pemphigus vulgaris was made after histologic and immunofluorescent examination of an intraoral deep incisional biopsy, with eosinophilic esophagitis also diagnosed during the initial upper gastrointestinal endoscopy. The association between pemphigus vulgaris and eosinophilic esophagitis in this case, although previously unreported, is explicable on the basis of dysregulation of desmoglein 1 (DSG1). This case report identifies a new clinical association that could help clinicians identify further such cases and provides insight into the pathogenesis of both conditions.
Subject(s)
Eosinophilic Esophagitis/complications , Eosinophilic Esophagitis/pathology , Pemphigus/complications , Pemphigus/pathology , Adolescent , Humans , MaleABSTRACT
OBJECTIVE: Early recognition and treatment of juvenile idiopathic arthritis (JIA) can prevent joint damage and minimize side effects of medication. The balance between proinflammatory and antiinflammatory mechanisms is known to be important in JIA, and we therefore investigated T cell subsets including Th cells, autoaggressive Th17 cells, and regulatory T cells (Treg), including a novel Treg subset in peripheral blood (PB) and synovial fluid (SF) of patients with JIA. METHODS: Fifty children with JIA were enrolled in our study. Frequency, phenotype, and function of T lymphocytes in PB and SF were characterized using flow cytometry. Migration capabilities of PB and SF cells were compared. RESULTS: Synovial T cells showed different phenotype and function compared with PB T cells, with an increased proportion of memory T cells, expression of CCR4, CCR5, CXCR3, interleukin 23R, and an increased ratio of Th17 to Treg. Although Treg were increased in SF compared with the PB, we found a significant decrease in the numbers of peptidase inhibitor 16 (PI16)+ Treg in active joints compared with peripheral blood. Coexpression of CCR4 and CCR6 was reduced on PI16+ Treg in PB and SF of patients with JIA compared with healthy children, however the ability of these cells to migrate toward their ligands was unaffected. CONCLUSION: This is a comprehensive characterization of novel PI16+ Treg and Th17 cells in matched blood and synovial fluid samples of patients with JIA. Despite an increased number of Treg within the inflamed joint, lower numbers of PI16+ Treg but high numbers of Th17 cells might contribute to the inability to control disease.
Subject(s)
Arthritis, Juvenile/immunology , Carrier Proteins/metabolism , Glycoproteins/metabolism , Synovial Fluid/immunology , T-Lymphocyte Subsets/metabolism , T-Lymphocytes, Regulatory/metabolism , Th17 Cells/metabolism , Adolescent , Arthritis, Juvenile/metabolism , Child , Child, Preschool , Female , Humans , Infant , Male , Synovial Fluid/metabolism , T-Lymphocyte Subsets/immunology , T-Lymphocytes, Regulatory/immunology , Th17 Cells/immunologyABSTRACT
OBJECTIVE: Neurologic involvement has been reported to occur in infants with neonatal lupus erythematosus (NLE), although the significance of these findings is unknown. The purpose of this study was to determine if hydrocephalus/macrocephaly is a manifestation of NLE. METHODS: Infants from the Hospital for Sick Children in Toronto, Canada whose mothers had anti-Ro antibodies were followed prospectively. A total of 87 infants were seen in the study period (1999-2004). The maternal autoantibody status of all infants was documented, and all infants underwent full clinical examination at each visit. RESULTS: Of the 87 infants, 47 had NLE. Five of the 47 infants with NLE and 2 of the healthy infants had hydrocephalus, resulting in a prevalence of 8.0% (95% confidence interval 4.0-15.0%) in the entire cohort. This is significantly higher than the prevalence in the general population of 0.048-0.081%. Head circumference measurements of infants in the Toronto cohort were largest between 12-24 months of age (Z score difference = 0.71, P = 0.008). CONCLUSION: We suggest that hydrocephalus and macrocephaly are manifestations of NLE and that infants born to mothers with anti-Ro antibodies should be carefully monitored for hydrocephalus as part of their routine physical examination.
