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November 1987

Familial Scleroderma in a Kindred With High Incidence of Autoimmune Disease: Correlation With HLA-A1/B8 Haplotype

Author Affiliations

Endocrine and Diabetes Clinic Oceanside, CA 92504; Department of Medicine University of California at Irvine Medical Center Orange, CA 92668

Arch Dermatol. 1987;123(11):1438-1440. doi:10.1001/archderm.1987.01660350032011

To the Editor.—  Familial scleroderma is a rare condition, and HLA typing in scleroderma is very limited. We describe herein two women with the HLA-A1/B8 haplotype in whom scleroderma also developed. One of the patients was hypothyroid. Both patients belonged to a family with a high incidence of Hashimoto's thyroiditis and hypothyroidism, insulindependent diabetes mellitus, early ovarian failure, and Addison's disease. Since HLA-B8 is well-known to be associated with autoimmune endocrine disease,1 the coexistence of HLA-A1 with HLA-B8 may have special significance in the development of scleroderma in patients with familial autoimmune polyendocrinopathies.

Report of Cases.—  The pedigree of the kindreds studied is illustrated in Fig 1. The two individuals affected by scleroderma were both women, Cl and C5.

Case I.—  Patient Cl was a 57-year-old woman in whom the diagnosis of scleroderma was established at the age of 38 years. Since that age, she suffered from dysphagia, bloating

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