Electrosurgical debulking of pretibial myxedema of the foot.

Pretibial myxedema or thyroid dermopathy constitutes dermal deposition of mucin, primarily hyaluronic acid and chondroitin sulfate. It is a manifestation of autoimmune thyroiditis, seen more in Graves disease than in Hashimoto thyroiditis. The time delay from treatment of hyperthyroidism to appearance of localized myxedema varies from one month to 16 years (mean 5.13 years). Despite a variety of therapeutic options, failure and relapse rates are high. Therapeutic options reported in the literature include compression, topical and intralesional corticosteroids, oral pentoxifylline, octreotide, rituximab, plasmapheresis, and high-dose intravenous immunoglobulin. We share our experience in two patients who were treated with electrosurgical debulking of selected longstanding myxedematous lesions, with one positive result and one negative result.

Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation.

BACKGROUND: Scleromyxedema is a rare chronic fibromucinous disorder that can have devastating clinical manifestations, including sclerosis of the skin with progressive pharyngeal and upper airway involvement, resulting in high mortality due to respiratory complications. Herein we describe a novel therapeutic approach. Because autologous hematopoietic stem cell transplantation is effective in other plasma cell proliferative disorders, it may be effective in this setting. OBSERVATIONS: We retrospectively evaluated 6 patients who were offered high-dose chemotherapy with stem cell rescue as treatment for scleromyxedema. One heavily pretreated patient was unable to mobilize stem cells. The remaining 5 patients mobilized stem cells and underwent successful transplantation. There was no treatment-related mortality. Hematologic responses were seen in 4 patients, including 2 complete remissions and 2 partial remissions, and all 4 had improvement in extracutaneous manifestations. All 4 patients subsequently had relapse of the monoclonal protein, and 3 developed skin relapses at 14, 37, and 45 months. CONCLUSIONS: High-dose chemotherapy with stem cell rescue is feasible for patients with scleromyxedema and, although not curative, offers durable remission in most patients. This therapy should be considered before treatment with alkylating agents or other treatments that could adversely affect the ability to collect stem cells.

Trastornos cutáneos y no cutáneos tratadoscon fotoféresis extracorpórea / Cutaneous and noncutaneous disorders treated with extracorporeal photopheresis

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Scleromyxedema (papular mucinosis): a surgical perspective.

Scleromyxedema (papular mucinosis) is a rare systemic disorder with a poor prognosis that produces severe aesthetic and functional disability. A patient is presented for whom the hitherto undescribed use of aggressive surgical intervention for palliation is applied. Methods are described, including a discussion of the trial-and-error evolution of the treatment. Results are shown that suggest that there is a place for surgical intervention in the treatment of the cutaneous changes produced by this disease.

Surgical excision of pseudotumorous pretibial myxedema.

Pretibial myxedema is a well-known clinical feature of autoimmune thyroid malfunction and is classically associated with Graves' disease. Its clinical course is frequently not related to the clinical control of the underlying thyroid disease. Treatment is primarily medical, consisting of oral and topical corticosteroids. We present a case of successful local excision of a pseudotumorous manifestation at the dorsum of the foot recalcitrant to multimodal medical and dermatological therapy.

Successful surgical treatment of severe pretibial myxoedema.

We report a 46-year-old man with severe and long-standing pretibial myxoedema, who responded well to local surgical treatment. This improvement has been maintained over a 12-month period, perhaps because of concomitant treatment with octreotide.

The rare presentation to the cosmetic and plastic surgeon of a patient with myxedema.

Myxedema results from hypofunction of the thyroid gland. Symptoms include dry skin, loss of and dryness of hair, mental apathy, drowsiness, and sensitivity to cold. Ocular complications associated with myxedema may be the symptoms that first prompt patients to seek a physician or cosmetic surgeon, however, though other symptoms may be present before eyelid myxedema occurs. The case reported here illustrates the value of a correct diagnosis and appropriate medical treatment, and demonstrates how surgical intervention to correct remaining eyelid problems can succeed when it is part of a comprehensive treatment plan.

The use of carbon dioxide laser in lichen myxedematosus.

Lichen myxedematosus is characterized by deposits of mucin in the dermis. Treatment can be systemic or local, depending on the clinical findings. We present a patient with pedunculated lesions on his nose, and a generalized thickening of his facial skin. The patient requested treatment of his nasal lesions. We elected to remove his nodules surgically using the carbon dioxide (CO2) laser. The technique is described and treatment options are reviewed.

Unusual locations of localized myxedema in Graves' disease. Report of three cases.

Three patients with Graves' disease had very unusual locations of localized myxedema. One patient had localized myxedema on the pretibial and shoulder areas after sodium iodide I 131 therapy. The second patient had localized myxedema on his neck, shoulders, and upper part of the back as well as on the pretibial area. The third patient had localized myxedema on the pinnae, also following sodium iodide I 131 therapy. After surgical removal followed by intralesional triamcinolone acetonide injections, the first patient's lesions recurred. The lesions in the second case did not respond to topical steroid cream. The third patient had a partial response to intralesional steroid injections.

Two cases of scleromyxedema.

We report two cases of scleromyxedema treated with melphalan (alkeran) and dermabrasion, both patients with central nervous system involvement. In the first case, herpetic encephalitis, possibly due to the immunoglobulin disturbance, preceded the skin changes. In the second case, impaired cerebral function was caused by a meningeoma. In the first case of short duration it seems as if progression of the disease has been arrested. In the second, of more than 26 years duration the skin changes have seemed unchanged, sclerotic for some years, though some improvement was noticed following dermabrasion.