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Lichen sclerosus premenarche: autoimmunity and immunogenetics
J Powell1, F Wojnarowska, S Winsey
1Departments of Dermatology and Transplant Immunology, Oxford Radcliffe Hospitals, The Churchill Hospital, Old Road, Headington, Oxford OX3 7LJ, U.K.
The British Journal of Dermatology
|March 29, 2000
Summary
Lichen sclerosus in children shows a strong link to HLA DQ7, a gene variant also associated with autoimmune diseases. Many affected children have a family history of autoimmunity, suggesting a genetic predisposition.
Area of Science:
- Immunogenetics
- Dermatology
- Pediatrics
Background:
- Lichen sclerosus is a common vulval disorder in children.
- Adults with lichen sclerosus show strong associations with autoimmune diseases and the HLA DQ7 gene.
- Previous studies have not investigated these associations in pediatric populations.
Purpose of the Study:
- To investigate HLA associations and autoimmune disease links in children with vulval lichen sclerosus.
- To compare findings in children with those reported in adult populations.
Main Methods:
- Human Leukocyte Antigen (HLA) tissue typing was performed on 30 children diagnosed with vulval lichen sclerosus.
- Autoimmune disease associations were assessed in the pediatric cohort and their families.
Main Results:
- HLA DQ7 was found in 66% of female children with lichen sclerosus, significantly higher than controls (31%).
- 16% of affected children were homozygous for DQ7, compared to 5% in controls.
- While only 4% of children had other autoimmune diseases, 56% of their parents or grandparents had a history of autoimmunity.
Conclusions:
- Pediatric vulval lichen sclerosus is strongly associated with the HLA DQ7 gene.
- A significant family history of autoimmune diseases suggests a genetic predisposition in early-onset lichen sclerosus.
- These findings highlight the importance of immunogenetic factors in childhood lichen sclerosus.