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Thyroid Dermopathy and Acropachy

      A 58-year-old man with a 9-year history of gradually worsening and disabling hand and feet swelling, with associated burning pain, is followed in Dermatology. He developed Graves disease 20 years ago, treated with radioactive iodine, and Graves ophthalmopathy treated with surgical decompression and tarsorrhaphy 8 years ago. He is a former smoker. Examination revealed disfiguring hypertrophic, firm, skin-colored overgrowth, and nonpitting edema of hands (Figure 1; Supplemental Figure, available online at http://www.mayoclinicproceedings.org) and feet (Figure 2).
      Figure thumbnail gr1
      Figure 1Hypertrophic skin-colored plaques and nodules of bilateral hands, dorsal view with nail clubbing, consistent with elephantiasis, thyroid dermopathy, and acropachy.
      Figure thumbnail gr2
      Figure 2Hypertrophic skin-colored plaques and nodules of bilateral feet, with nail clubbing and serous crusting of proximal second toes bilaterally.
      He had normal thyroid hormone levels. Previous biopsy from the calf was consistent with pretibial myxedema. He was diagnosed with severe thyroid dermopathy and acropachy. He has been treated with a combination of compression, topical and intralesional corticosteroid, intralesional hyaluronidase, intravenous rituximab, localized radiotherapy, and oral gabapentin, with limited or short-term efficacy.
      Thyroid dermopathy occurs with autoimmune thyroid disease, particularly Graves disease, and is thought to be caused by several factors including autoimmunity, limb dependency, mechanical injury, and tobacco use.
      • Fatourechi V.
      Thyroid dermopathy and acropachy.
      Approximately 20% of patients with dermopathy also have acropachy with clubbing, digital swelling or periosteal reaction, and dermopathy is almost always associated with ophthalmopathy.
      • Fatourechi V.
      • Ahmed D.D.
      • Schwartz K.M.
      Thyroid acropachy: report of 40 patients treated at a single institution in a 26-year period.
      Although dermopathy typically affects pretibial region with firm, nonpitting edema, it may also affect acral sites and progress to lymphedema and elephantiasis in severe forms.
      • Fatourechi V.
      • Ahmed D.D.
      • Schwartz K.M.
      Thyroid acropachy: report of 40 patients treated at a single institution in a 26-year period.
      ,
      • Guerrero C.
      • Pittelkow M.R.
      Images in clinical medicine: thyroid ophthalmopathy, dermopathy, and acropachy.
      Many treatments have been reported with variable efficacy. These include topical, intralesional, and systemic corticosteroids; compression therapy; radiotherapy; hyaluronidase; tumor necrosis factor inhibitors; tocilizumab; rituximab; and teprotumumab (insulin-like growth factor-1 receptor blocking antibody).
      • Kraus C.N.
      • Sodha P.
      • Vaidyanathan P.
      • Kirkorian A.Y.
      Thyroid dermopathy and acropachy in pediatric patients.
      • Bartalena L.
      • Fatourechi V.
      Extrathyroidal manifestations of Graves' disease: a 2014 update.
      • Kotwal A.
      • Turcu A.F.
      • Sonawane V.
      • et al.
      Clinical experience with rituximab and intravenous immunoglobulin for pretibial myxedema: a case series.
      Thyroid dermopathy and acropachy are important to recognize as severe signs of autoimmune thyroid disease and thyroid ophthalmopathy.

      Potential Competing Interests

      The authors have no competing interests.

      Acknowledgments

      We are grateful to the Appignani Lichen Planus Benefactor Gift and the British Association of Dermatologist’s Geoffrey Dowling Fellowship for supporting Dr Xie’s visiting research fellowship.

      Supplemental Online Material

      Figure thumbnail figs1
      Supplemental Figure 1Hypertrophic skin-colored overgrowth of bilateral hands, palmar view.

      References

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        Images in clinical medicine: thyroid ophthalmopathy, dermopathy, and acropachy.
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        Extrathyroidal manifestations of Graves' disease: a 2014 update.
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        Clinical experience with rituximab and intravenous immunoglobulin for pretibial myxedema: a case series.
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