My 15 year old daughter, who has type 1 diabetes and anemia, has been diagnosed with eosinophilic fasciitis. This was diagnosed by doing a biopsy, and I am told it is an autoimmune disease that belongs in the family of scleroderma. She has had diabetes since she was a year old, was recently started on an insulin pump, and her hemoglobin A1c is 8.9%. Her free T-4 level was low with a normal TSH.

She goes to an adult endocrinologist who he said that if she were hypothyroid, her TSH level would be elevated, but I have read that children can have normal TSH and still be hypothyroid or what they termed at central hypothyroidism. Could you please explain this to me? Some of her symptoms are the same for hypothyroidism.

The treatment for eosinophilic fasciitis is either a corticosteroid or low dose methotrexate. Which would be the lesser of two evils in considering the diabetes and everything going on with my daughter?

I assume that your daughter has type 1A (autoimmune) diabetes, and that this may have been confirmed at the time of diagnosis by a positive antibody test. In any case, it is by far the most common form of diabetes in childhood in Caucasian families in North America. This condition may quite often be accompanied by other autoimmune states of which the most common is hypothyroidism in a syndrome that is now recognised as the Autoimmune Polyglandular Syndrome Type II The diagnostic test for this, as the endocrinologist said, is a raised TSH. TSH is a pituitary hormone, and, in some very rare conditions where this gland at the base of the brain is damaged, TSH production is reduced so that what you have called central hypothyroidism can result. This form of hypothyroidism has no basis in autoimmunity.

Several forms of fasciitis have an immune basis and are included in APS II, but eosinophilic fasciitis is not listed as one of them, perhaps because it is so rare and perhaps because the link to autoimmunity is still rather tenuous. See Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. 1997 Nov;9(6):562-70.

I have personally no experience in this condition, but both steroids and methotrexate are reported as standard therapy.Steroids may make the control of the diabetes a little more difficult, and that is perhaps why the doctor suggested methotrexate.

DOB
Additional comments from Dr. Stuart Brink:

This is a very rare situation. Central hypothyroidism usually occurs with hypothalamic or pituitary disease such as a tumor or radiation therapy to the region. She could have what is called sick euthyroid syndrome and this does not need to be treated with thyroid hormone. Sequential thyroid functions should help as might a TBG level.

Methotrexate might be slightly less toxic than steroids but this should be decide by the treating physicians. If you use steroids, the insulin dose will dramatically raise within 8-12 hours of the first dose. On an insulin pump, this would correspond to a significant increase in basal insulin so you should stay in close contact with your diabetes team so that they can help make such adjustments. Both should be manageable.

SB