Pretibial myxedema in a euthyroid patient.

Pretibial myxedema (PM) is a rare extrathyroidal manifestation of Graves' disease (GD), usually during the hyperthyroid state, coexisting with orbitopathy. We describe a rare case of a biopsy-proven PM in a euthyroid patient, without history of GD or Hashimoto's thyroiditis. Assessment of commonly reported thyroid autoantibodies, such as thyroid peroxidase and thyroglobulin autoantibodies, thyroid stimulating immunoglobulins and thyroid binding inhibitory immunoglobulins, was negative. Resolution of skin pathology was achieved after topical application of corticosteroids and was sustained 1 year later.

Hyperhomocysteinemia in patients with stasis dermatitis and ulcer: a novel finding with important therapeutic implications.

BACKGROUND: Although homocysteine (Hcy) has profound effects concerning vascular lesions and thrombosis, it has not previously been investigated in patients with stasis dermatitis and ulcer. OBJECTIVE: To evaluate plasma Hcy levels in patients with stasis dermatitis and ulcer. METHODS: A total of 25 patients (17 male, eight female; mean age of 36 +/- 5.97 years) with stasis dermatitis, 40 patients with stasis ulcer (27 male, 13 female; mean age of 38.5 +/- 7.96 years) and 35 healthy control individuals (25 male, 10 female; mean age of 36.9 +/- 6.49 years) were included in this study. Patients taking vitamin supplements or patients with folic acid or vitamin B12 deficiency, diabetes mellitus, chronic hepatitis, renal failure and chronic alcoholism which might affect Hcy levels were excluded from the study. RESULTS: Hcy levels were found to be elevated in patients with stasis dermatitis (p = 0.00) and stasis ulcer (p = 0.00) compared to healthy controls with a median (range) of 19.1 micromol/l (15-28), 18.98 micromol/l (15-29), and 8.1 micromol/l (5-12.2), respectively. There was no statistically significant difference in Hcy level in patients with stasis dermatitis and ulcer (p = 0.877). CONCLUSION: As hyperhomocysteinemia can be treated by selected vitamin supplementation even in patients with normal serum vitamin concentrations, ulcer formation might be prevented by lowering Hcy in the clinical stage of stasis dermatitis.

Cutaneous involvement as the first manifestation in a case of T-cell prolymphocytic leukaemia.

Mature T-cell malignancies of extracutaneous origin are rare disorders. T-cell prolymphocytic leukaemia (T-PLL) is the most common form of all mature T-cell leukaemias in adults. Secondary skill involvement by T-PLL has been reported in 25% of patients. A case of T-PLL which presented with cutaneous infiltration mimicking a cellulitis-like lesion resistant to antibiotic therapy is described. The diagnosis of T-PLL was subsequently fully supported by the clinical, laboratory and cytological findings, as well as by the immunophenotypic study of the skin biopsy. The present case stresses the importance of accurate evaluation of skin lesions in the diagnosis of some haematological conditions and gives additional information about T-PLL such as a previously non-reported cytogenetic abnormality [t(6;6)] and lack of cutaneous lymphocytic-associated antigen expression.

Use of a durometer to measure the degree of skin induration in lipodermatosclerosis.

BACKGROUND: Chronic lipodermatosclerosis is characterized by indurated skin on the medial aspect of the leg and is common around venous ulcers. The severity of induration of lipodermatosclerosis has been associated with poor ulcer healing. Clinical assessment of lipodermatosclerosis presently relies on a clinical skin severity score adapted from studies of patients with systemic sclerosis. OBJECTIVE: It would be desirable for prognostic reasons to develop an objective method for measuring skin hardness in lipodermatosclerosis. METHODS: The degree of skin induration at the midpoint between the upper and lower margin of lipodermatosclerosis in 30 sequential nonselected patients with lipodermatosclerosis was assessed by a blinded observer's clinical score and by quadruplicate determinations with a hand-held type O durometer. Skin induration on the medial aspect of the leg was similarly measured in five normal volunteers. Transcutaneous oxygen pressure was measured at the same sites. RESULTS: A direct linear relation (r = 0.962) was found between skin severity scores and durometer readings (p < 0.01). A clinical skin score of 2 reflected a higher durometer reading compared with a skin score of 1 (p = 0.0016) and, similarly, higher durometer readings were found in skin score of 3 compared with score 2 skin (p = 0.0093). Transcutaneous oxygen pressure was uniformly reduced in lipodermatosclerosis (p < 0.02). CONCLUSION: The durometer is a reliable instrument for measuring skin hardness in patients with lipodermatosclerosis. It may be used to test the prognostic value of lipodermatosclerosis on ulcer healing.

IgA anticardiolipin antibodies associated with Henoch-Schönlein purpura.

Henoch-Schönlein purpura is associated with the deposition of immune complexes containing IgA. The nature of the antigen in these immune complexes is uncertain but in some reported cases has included autoantigens such as IgA rheumatoid factor and IgA antineutrophil cytoplasmic antibody. We report the finding of an IgA class anticardiolipin antibody in a 51-year-old patient with Henoch-Schönlein purpura. A potential role for IgA autoantibodies in Henoch-Schönlein purpura needs to be further explored.

TSH and TSH receptor antibody-binding sites in fibroblasts of pretibial myxedema are related to the extracellular domain of entire TSH receptor.

The role of TSH receptor antibodies in the pathogenesis of pretibial myxedema is still unclear. This study was designed to determine whether patients with pretibial myxedema had higher serum titers of TSH receptor antibodies, and whether there were TSH and TSH receptor antibody-binding sites on plasma membranes of fibroblasts derived from the skin of pretibial myxedema. If there were, were the binding sites similar to the TSH receptor? The TSH receptor antibodies were determined with radioreceptor assay in 20 normal subjects, 18 hyperthyroid Graves' disease patients without ophthalmopathy, 26 hyperthyroid Graves' disease patients with ophthalmopathy, and 11 patients with pretibial myxedema associated with Graves' ophthalmopathy. TSH and TSH receptor antibody-binding sites were studied on plasma membranes of fibroblasts cultured from the skin of pretibial myxedema with radioreceptor assay. RNA was also extracted from the fibroblasts of pretibial myxedema and reverse transcribed using random primers as the primers for cDNA synthesis. The resulting cDNAs were subjected to amplification by polymerase chain reaction with the use of a set of primers spanning the 5' region (+256/+275 and +616/+635) and the 3' region (+1819/+1838 and +2405/+2424) of the TSH receptor cDNA (+1 transcription start codon). They were further identified by Southern blot hybridization, with the probe spanning the 5' region (+272/+612) and the 3' region (+1908/+2268) of the TSH receptor cDNA (+ 1 transcription start codon), and sequencing. The results showed that patients with pretibial myxedema had higher titers of TSH receptor antibodies in the serum. TSH and TSH receptor antibody-binding sites were present on plasma membranes of fibroblasts derived from the skin of pretibial myxedema patients and related to the extracellular domain of the TSH receptor. These data suggest a common antigenic site in the skin and in the thyroid as a putative target for TSH receptor antibodies or lymphocytes of Graves' disease.

[Cyclosporin A therapy in pyoderma gangraenosum]. / Cyclosporin-A-Therapie bei Pyoderma gangraenosum.

A 33-year-old woman with pyoderma gangrenosum was treated with cyclosporine A. Cyclosporine A is a valuable drug for the treatment of recalcitrant pyoderma gangrenosum. Its mechanism of action is discussed. The numerous side-effects require close monitoring of cyclosporine A blood levels and several other parameters.

The normocomplementemic urticarial vasculitis syndrome--report of a case and response to colchicine.

A patient with a 15-year history of urticarial vasculitic rashes and chronic vasculitic skin ulceration has been followed at our Connective Tissue Disease Clinic for the past five years. Serum complement levels have been persistently normal. She has been unresponsive to a variety of medications including steroids, dapsone and hydroxychloroquine. She had a dramatic response to colchicine 500 micrograms daily with total clearing of the urticarial vasculitic rash.

[Immunopathogenetic, endocrinologic and therapeutic problems of EMO syndrome. Observations based on a case report]. / Immunpathogenetische, endokrinologische und therapeutische Probleme des EMO-syndroms. Betrachtungen anhand einer Krankenbeobachtung.

The EMO-syndrome (thyroid acropachy) consists of the triad of exophthalmus, circumscribed pretibial myxedema and hypertrophic osteoarthropathy. The clinical picture is seen in thyroid disorders, as a rule occurring in hyperthyroidism, rarely in hypothyroid or euthyroid states. Our 47 year old female patient showed a hyperthyroidism in the beginning. The thyrostatic treatment resulted in a euthyroid stage. Serum antibodies against microsomal thyroid gland antigen with high titers were detected. The immunosuppressive treatment resulted in a titer reduction, but not in an improvement of the disease. The pathogenetical importance of the thyroid stimulating immunoglobulins and the role of aggressive and destructive antibodies against thyroid gland for the development of EMO-syndrome are discussed.

Sweet's syndrome. Report of a case.

A case of relentlessly recurrent, violently inflammatory, and extremely painful Sweet's syndrome of twenty-three years' duration is presented. For many years lesions were often solitary, and frequently localized to the left adductor thigh. This led to the misdiagnosis of the condition as recurrent herpes simplex. For twenty-three years the disease was localized to the left lower extremity. Recurrences became more protracted during the past two years. The most prominent symptom was lesional pain, and recently this could not be relieved with narcotic analgesics. Lesions were asymmetric, bright red, raised, painful and had sharp borders. The largest lesion measured 18 cm in diameter. Response to systemic steroids was prompt and dramatic, and healing occurred without scarring.

Pretibial myxoedema: stimulation of mucopolysaccharide production of fibroblasts by serum.

We have shown that sera from normal individuals and from patients with pretibial myxoedema contain a factor which simulates mucopolysaccharide biosynthesis in normal human skin fibroblasts cultured in vitro. This factor was present in larger amounts in sera of patients with pretibial myxoedema. The role of growth stimulating factors in serum is reviewed and a hypothesis is put forward that the fibroblast stimulating factor is somatomedin and that its presence in increased amounts in thyroid disease may lead to pretibial myxoedema.

[Studies on the radioreceptor assay of TSH: the properties of TSH-binding inhibitor immunoglobulins (TBII) in patients with Graves' disease (author's transl)].

In the radioreceptor assay system for TSH, serum immunoglobulin G (IgG) from some patients with Graves' disease has been shown to inhibit the binding of labelled TSH to its receptor sites. In order to clarify the properties of these TSH-binding inhibitor immunoglobulins (TBII) in patients with Graves' disease, TBII were measured in sera from 31 untreated and 51 131I-treated patients, and their relation to clinical and laboratory findings was studied. TBII were detected in 18 (60%) out of 31 patients with untreated Graves' disease. TBII levels in these patients correlated well with thyroidal 99mTc uptake at 30 min and also with the grade of epithelial hyperplasia of thyroid follicles. There was no significant correlation between TBII and serum T3, serum T4, free T4 index, antibody titers against thyroglobulin and microsomes, or association of exophthalmos. There were many patients with Graves' disease whose sera contained high TBII levels but no detectable bioassayable thyroid-stimulating activity (LATS), and in these patients a close correlation was observed between serum levels of TBII and bioassayable LATS-protector activity. In patients with Graves' disease who had been treated by 131I from 5 to 17 years before, the incidence of TBII was very low at 20% (10/51). All except two cases having TBII were found to be still thyrotoxic. Thus, TBII were detected in 8 out of 10 thyrotoxic patients and in only 2 out of 18 euthyroid and none of 23 hypothyroid patients. These findings suggest that TBII in patients with Graves' disease were in close association with human thyroid stimulating activity, and that TBII might be useful as an indicator for checking the effectiveness of the treatment.

Stimulation of fibroblast biosynthetic activity by serum of patients with pretibial myxedema.

Skin fibroblasts from the shoulder and lower extremities of normal individuals, as well as from patients with pretibial myxedema (PTM) were grown in culture. When cells reached the monolayer stage, they were labeled with 3H-glucosamine and tested for hyaluronic acid synthesis in the presence of either serum from PTM patients or normal human serum. All the fibroblasts from the pretibial area synthesized 2 to 3 times more hyaluronic acid when incubated with PTM sera than when incubated in normal human serum. Fibroblasts cultured from skin of the back or prepuce did not respond to PTM sera. This heat-stable, protease-sensitive, and dialyzable, fibroblast-stimulating factor is not a 7S gamma-globulin. The enhanced sensitivity to PTM sera exhibited by fibroblasts from the lower extremities may explain why the lesions in this disease are restricted primarily to that area.