1/220. Recognizing the faces of hypothyroidism. physicians may not recognize hypothyroidism if they rely on the stereotypical picture of the disorder. The age of the patient, stage of the disease, and other illnesses or conditions such as pregnancy can change the clinical presentation. The signs and symptoms of hypothyroidism are remarkably diverse. Instead of a single picture, physicians need a mental gallery. ( info) |
| A case of cardiac tamponade secondary to primary myxedema is described. The nature of the patient's pericardial fluid and clinical course compared with other cases in the literature is reviewed. The patient had no recurrence of cardiac tamponade. Complete resolution of the pericardial effusion occurred 10 months followint initial pericardiocentesis and L-thyroxine therapy. ( info) |
| scleromyxedema is a disorder characterized by a typical rash due to the accumulation of mucin in the dermis. It is always associated with a monoclonal protein in the serum and can have a wide variety of systemic manifestations. We describe a 40-year-old woman who had scleromyxedema associated with a monoclonal G lambda protein. Severe systemic symptoms included fatigue, esophageal dysmotility, and myopathy. Symptoms resolved completely with oral prednisone therapy, and she remained in clinical remission 24 months after use of prednisone was discontinued. scleromyxedema is commonly treated with alkylating agents, which have been associated with pronounced morbidity and mortality. We suggest that oral corticosteroid therapy may be a reasonable initial choice for treating this disease and that alkylating agents be reserved for corticosteroid-refractory disease. ( info) |
| Eccrine sweat duct proliferations may be found in various inflammatory and neoplastic skin lesions. We report a patient with scleromyxedema with extensive proliferations of intradermal sweat ducts. Three-dimensional reconstruction demonstrated extensive coiling and branching of the sweat ducts leading into cystic lacunae. In contrast to the basal cell carcinoma that had grown within the scleromyxedematous skin, the ducts close to the lumen stained positive for carcinoembryonic antigen and could therefore be differentiated from basal cell carcinoma. In micrographically controlled surgery of cutaneous epithelial tumors that are located in chronically inflamed skin, such sweat gland proliferations have to be considered as differential diagnosis. ( info) |
5/220. A rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema: case report. The development of pituitary tumours as a consequence of primary target organ failure is rare. We report here a rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema. This case presentation suggested the importance of detailed endocrine investigation and repeated magnetic resonance imaging for the differential diagnosis of pituitary enlargement to avoid unnecessary surgery. ( info) |
| The authors monitored CSF findings for over 5 months in a patient with a fatal case of scleromyxedema and two episodes of encephalopathy. During both encephalopathy episodes, CSF protein and immunoglobulin g (IgG) levels were elevated without an increased IgG index or IgG synthesis rate. A CSF-dominant increase in the concentration of interleukin-6 (IL-6) also was noted during encephalopathy. These findings suggest a disruption of the blood-brain barrier and that IL-6 may play some role in mediating the encephalopathy. OFF ( info) |
7/220. scleromyxedema is a scleroderma-like disorder and not a coexistance of scleroderma with papular mucinosis. We present four cases of scleromyxedema with scleroderma-like cutaneous changes mimicking systemic sclerosis and stress the importance of their differentiation from true scleroderma. scleromyxedema should be recognized as an entity since it differs from scleroderma in the pathogenesis, histopathology of cutaneous lesions, type of visceral involvement (if present), frequent association with paraproteinemia, the course and prognosis. ( info) |
8/220. Factors associated with mortality of myxedema coma: report of eight cases and literature survey. High-dose thyroid hormone replacement has been recommended for treatment of myxedema coma (MC) while questions of safety of the therapy and of efficacy of low-dose thyroid hormone replacement have not been systematically addressed. We treated 8 patients with MC in a period of 18 years, the first 3 with high-dose intravenous injections of levotriiodothyronine (LT3) and the other 5 patients with a smaller amount of either LT3 or levothyroxine (LT4). Two of the first 3 patients died of pneumonia and the other 5 recovered despite pulmonary abnormalities at the outset. To find factors associated with fatal outcome after treatment, the medline database was searched for MC cases with data of thyroid hormone replacement and outcome within 1 month of therapy. Clinical data for our 5 patients and 82 cases from the medline search were pooled and factors associated with mortality were sought among age, gender, presence of cardiac or pulmonary complications, and doses of thyroid hormone by multiple logistic regression analysis. It revealed that greater age, cardiac complications, and high-dose thyroid hormone replacement (LT4 > or = 500 microg/d or LT3 > or = 75 microg/d) were significantly associated with a fatal outcome within 1 month of treatment. Elderly MC patients can be treated with low-dose hormone replacement. A bolus of 500 microg LT4, especially by mouth or via nasogastric tube, appears to be tolerated by younger patients (< 55 years) without cardiac complication. The conclusion remains to be confirmed in more patients. ( info) |
9/220. exophthalmos, pretibial myxedema, osteoarthropathy syndrome associated with papillary fibroelastoma in the left ventricle. EMO syndrome, a rare complication of Graves' disease, exhibits exophthalmos, pretibial myxedema, and osteoarthropathy. The presence of functional thyrotropin receptors (TSHR) in adipocytes and osteoblasts, both of which we have recently observed, may be related to these extrathyroidal manifestations of Graves' disease. In addition, the expression of TSHR in the heart has recently been reported. We describe here a patient with Graves' disease exhibiting EMO syndrome with a papillary fibroelastoma in the left ventricle. Pathological examinations showed that the fibroelastoma contained alcian blue-stained mucinous materials that were also observed in the subcutaneous tissue of pretibial myxedema. ( info) |
10/220. scleromyxedema: successful treatment of cutaneous and neurologic symptoms. scleromyxedema is a rare systemic disorder characterized by cutaneous sclerosis and papulosis, accompanied by deposition of mucin in the skin and other organs. We describe a case of scleromyxedema in a 62-year-old man. The cutaneous symptoms of the disorder were preceded by episodes of acute central nervous system dysfunction that included mental confusion, hemiparesis, tremor, and migraine. As the cutaneous symptoms progressed, the patient experienced persistent confusion and difficulty concentrating. Therapy with melphalan and plasmapheresis led to complete resolution of the cutaneous symptoms as well as near-resolution of the neurologic symptoms. This is the first report to describe the successful treatment of the cutaneous symptoms of scleromyxedema accompanied by reversal of chronic neurologic dysfunction. ( info) |