Associated Disorders

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Dermatitis herpetiformis This is characterized by an extremely pruritic papulovesicular eruption, which usually occurs symmetrically on the elbows, knees, buttocks, and back. About 80% of patients with dermatitis herpetiformis have small intestine histology indistinguishable from celiac sprue. The diagnosis is established by skin biopsy demonstrating granular IgA deposits in areas of normal appearing skin. A majority of patients with the skin lesion who undergo small bowel biopsy have intestinal mucosal changes of celiac disease. The skin lesions, as well as small bowel histology, improve on a gluten-free diet. Dapsone is an effective short-term treatment for dermatitis herpetiformis; however, it does not have any impact on management of small bowel enteropathy. Also, those with dermatitis her-petiformis who are not compliant with the gluten-free diet are at higher risk for malignancy, as are those with celiac disease.

Celiac disease has also been associated with other autoimmune as well as nonautoimmune disorders. It has been reported that the longer there is exposure to gluten in patients with celiac disease, the greater the occurrence of other autoimmune diseases. There is evidence for a strong association between type 1 diabetes and celiac disease. About 8% of patients with type 1 diabetes have the characteristic features of celiac sprue on small bowel biopsy. When the two diseases coexist, 90% have the diagnosis of diabetes before celiac disease. Among the symptoms that may be suggestive of coexisting celiac disease, in addition to those considered classical for celiac disease, are delayed puberty, hypertransaminasemia, anemia, iron deficiency, arthralgias, dental enamel defects, hypoglycemia, and unexplained reduction in insulin requirements. Treatment with a gluten-free diet may improve diabetic control and decrease the occurrence of hypoglycemia episodes.

There is also a strong association between selective IgA deficiency and celiac sprue. Studies including adults and children in Ireland and Italy reported the frequency of selective IgA deficiency in celiac sprue to be about 2%, and 5-11% of IgA-deficient individuals have celiac disease.

There is a strong association between Down's syndrome and celiac disease. Individuals with Down's syndrome and celiac disease more commonly have gastrointestinal manifestations such as intermittent diarrhea, failure to thrive, anemia, and low serum iron and calcium. The prevalence of celiac disease in patients with Down's syndrome varies between 5 and 12%. An increased prevalence of celiac sprue has also been reported in individuals with Turner's syndrome and Williams syndrome.

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