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      Dermopathy of Graves’ disease: Clinico-pathological correlation

      case-report

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          Abstract

          Dermopathy of Graves’ disease is a classical, but uncommon extrathyroidal manifestation of Graves’ disease. The images of a typical case of dermopathy of Graves’ disease are presented along with clinico-pathological correlation.

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          Pretibial myxedema: pathophysiology and treatment options.

          Pretibial myxedema or localized myxedema or thyroid dermopathy is an autoimmune manifestation of Graves' disease. It also occasionally occurs in Hashimoto's thyroiditis. Lesions of thyroid dermopathy are usually asymptomatic and have only cosmetic importance. Advanced forms of dermopathy are associated with elephantiasis or thyroid acropachy. Almost all cases of thyroid dermopathy are associated with relatively severe ophthalmopathy. Usually ophthalmopathy appears first and dermopathy much later. All patients with localized myxedema have high serum concentrations of thyroid-stimulating hormone receptor antibodies, indicating the severity of the autoimmune condition. Occurrence of thyroid dermopathy in areas other than pretibial skin indicates a systemic process. Similar to Graves' ophthalmopathy, thyroid-stimulating hormone receptors in the connective tissue may be the antigen responsible for the immune process. Both humoral and cellular immune mechanisms are involved in the stimulation of fibroblasts and the production of large amounts of glycosaminoglycans. Localization in the pretibial area relates to mechanical factors and dependent position. Diagnosis of thyroid dermopathy is based on signs and typical pretibial skin lesions in association with a history of Graves' hyperthyroidism and ophthalmopathy. In some cases, skin biopsy is needed for confirmation. The lesions are usually mild and are overshadowed by more symptomatic ophthalmopathy. Most cases of thyroid dermopathy do not require any therapy. In mildly severe symptomatic cases and when there is cosmetic concern, topical corticosteroids applied under occlusive dressing are beneficial. In more severe cases, systemic immunomodulation may be necessary; however, conclusive evidence for long-term efficacy of these modalities is lacking. When significant edema and elephantiasis are present, local compressive therapy may have added benefit. In mild cases that do not require treatment, 50% of patients achieve complete remission after several years. Severe cases that receive topical corticosteroids or other therapies do not have a better outcome than untreated milder cases. Current treatment modalities for thyroid dermopathy and acropachy are at best palliative. Better and safer means of immunomodulation are needed.
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            Dermopathy of Graves' disease (pretibial myxedema): long-term outcome.

            Little is known about the long-term outcome of patients with thyroid dermopathy, an extrathyroidal manifestation of Graves' disease. Also, it is not known to what degree treatment promotes remission of the lesions. The present report supplies information on the natural course of mild, untreated and severe, treated thyroid dermopathy. In this study, we report on the outcomes of 178 patients seen at our institution between January 1969 and November 1995 with thyroid dermopathy who were followed up for an average of 7.9 yr. Nonpitting edema was the most prevalent form of dermopathy (43.3%), and the pretibial area was the region most commonly involved (99.4%). The majority of patients with dermopathy had ophthalmopathy (97.0%). Topical corticosteroids were the most commonly used treatment (53.9%). Patients with milder forms of dermopathy (40.4%) did not receive any therapy for dermopathy. Twenty-six percent of the patients experienced complete remission, 24.2% had moderate improvement (partial remission), and 50.0% had no or minimal improvement of their dermopathy at last follow-up. Patients who did not receive therapy experienced a significantly (P = 0.03) higher rate of complete remission (34.7%) than those who received local therapy (18.7%), although the combined complete and partial remission rates were not significantly different for the treated and untreated groups (P = 0.3). However, the treated and untreated groups were not comparable because our practice is to use therapy for more extensive and severe cases. All five cases of elephantiasis were in the treatment group and were less likely to have remission because of the severity of their skin condition. Patients receiving treatment were more likely to have dermatologic consultation and histologic diagnosis (P < 0.001). The beneficial effect of topical corticosteroid therapy on long-term remission rates remains to be determined.
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              Thyroid acropachy: report of 40 patients treated at a single institution in a 26-year period.

              Thyroid acropachy is an extreme manifestation of autoimmune thyroid disease. It presents with digital clubbing, swelling of digits and toes, and periosteal reaction of extremity bones. It is almost always associated with ophthalmopathy and thyroid dermopathy. During a 26-yr period at our institution, of 178 patients with thyroid dermopathy, 40 had acropachy. Clubbing associated with thyroid dermopathy (pretibial myxedema) was seen in 35 patients. Clubbing usually was not a patient complaint and was noted only by clinical observers. Four of eight patients with hand and extremity radiographs had periosteal reaction. Seven had associated extremity and joint pain; this pain was absent at long-term follow-up. Half of the patients required systemic corticosteroid therapy, 53% required transantral or transfrontal orbital decompression for severe ophthalmopathy, and 18% had the elephantiasic form of dermopathy. Cigarette-smoking rates were 81% for women and 75% for men (mean, 28 pack-years). All 13 patients who had thyroid-stimulating Ig measurement had high titers. Long-term follow-up (median, 12.5 yr) revealed that acropachy was not a complaint in follow-up visits or questionnaires. The data suggest that thyroid acropachy is an indicator of severity of ophthalmopathy and dermopathy. It is a source of clinical concern only if dermopathy is persistent and severe.
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                Author and article information

                Journal
                Indian J Endocrinol Metab
                Indian J Endocrinol Metab
                IJEM
                Indian Journal of Endocrinology and Metabolism
                Medknow Publications & Media Pvt Ltd (India )
                2230-8210
                2230-9500
                May-Jun 2012
                : 16
                : 3
                : 460-462
                Affiliations
                [1] Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
                [1 ] Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
                Author notes
                Corresponding Author: Dr. Sagili Vijaya Bhaskar Reddy, Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow – 226014, Uttar Pradesh, India. E-mail: svbreddy1976@ 123456yahoo.co.in
                Article
                IJEM-16-460
                10.4103/2230-8210.95714
                3354863
                22629522
                4fc861d6-e944-43e6-8b8b-3e22f6ef6c58
                Copyright: © Indian Journal of Endocrinology and Metabolism

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Case Report

                Endocrinology & Diabetes
                clinico-pathological correlation,dermopathy of graves’ disease,graves’ disease

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