[Scleroderma. Which tests should be performed?].
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Antibodies, Antinuclear↗
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[L-tryptophan-induced eosinophilia-myalgia syndrome with features of diffuse fasciitis with eosinophilia].
Two female patients developed localized scleroderma on the trunk and the thighs after oral ingestion of L-tryptophan for some years. Both patients reported acute progressive myalgia and weakness of the proximal parts of the extremities. On laboratory evaluation, the leucocyte count was approximately 20,000/mm3, with 38% blood eosinophils in one patient and 53% in the other. The ESR was slightly elevated; electrophoresis and muscle enzymes were normal. Skin and muscle biopsies revealed characteristic features of diffuse fasciitis with eosinophilia. High-dose glucocorticoid therapy resulted in a rapid normalization of the ESR and blood eosinophilia, whereas the scleroderma showed little improvement. The diffuse edema observed in one patient receded within a few days. A correlation between oral ingestion of L-tryptophan and the eosinophilia-myalgia syndrome has been reported recently, and the present case reports must be discussed in the light of this observation. Both patients developed a tryptophan-induced scleroderma-like illness resembling diffuse fasciitis with eosinophilia (Shulman's syndrome).
Eosinophilia↗
[Hemostasis in patients with systemic skin diseases].
Examinations of the hemostasis system parameters in patients with scleroderma, psoriatic arthritis, malignant lymphoma of the skin have revealed a significant elevation of fibrinogen concentration, acceleration of blood clotting time, increased prothrombin consumption in all the patients. Elevation of the prothrombin index was detected in the patients with scleroderma and psoriatic arthritis. A direct relationship between the disease severity, size of the involvement of the skin integument, inflammation activity in the joints, and severity of hemostasis impairment has been revealed.
Adolescent↗
Elevated plasma histamine levels in systemic sclerosis (scleroderma).
Systemic sclerosis is characterized by excessive deposition of collagen and other matrix proteins in the skin and internal organs. One hypothesis supports fibroblast stimulation for production of excess amounts of collagen by factors present in the blood or released by cells composing inflammatory tissue infiltrates. Increased numbers of mast cells are present in the involved skin of patients with systemic sclerosis, and histamine has been thought to be a possible mediator of fibrosis in this and other fibrotic conditions. We therefore measured plasma histamine levels in 32 patients with systemic sclerosis and found elevated levels in 18 patients (56%). Elevated plasma histamine levels were more common in patients with diffuse disease (74%), in contrast to limited disease (31%). The degree of clinical activity and the duration of disease could not be correlated with histamine levels.
Adult↗
[Characteristics of the course of scleroderma in diabetes mellitus].
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Adolescent↗
A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma).
Clinicopathologic correlations of myocardial fibrosis were examined in 54 autopsied patients with scleroderma and 54 age and sex matched autopsy controls. Thirty eight (70%) of the patients with scleroderma had myocardial fibrosis compared to 20 (37%) of the controls (p less than 0.005). There was no significant difference in the prevalence of contraction band necrosis in the patients with scleroderma (22%) compared to controls (17%). Patients with scleroderma with left ventricular dysfunction in the absence of other causative factors clinically had a greater prevalence of both advanced myocardial fibrosis (60%) and contraction band necrosis (40%) than did the other patients with scleroderma or the controls. We conclude that patients with scleroderma with the greatest likelihood of advanced myocardial fibrosis can be identified clinically, and their findings are consistent with the presence of microvascular coronary vasospasm, a "myocardial Raynaud's phenomenon."
Adult↗
Chromosome studies in scleroderma with consideration of anticentromere antibody status and assessment of possible in vitro clastogenic activity.
The constitutional karyotype and frequency of sporadic chromosome abnormalities in peripheral blood leukocytes from 30 scleroderma patients and 15 normal controls were studied. Fifteen of the scleroderma patients were positive for the anticentromere antibody (ACA) and 15 were negative. The constitutional karyotype of all patients and controls were normal. No statistically significant difference in sporadic chromosome abnormalities was detected among the two groups of scleroderma patients compared with the control group. The possibility of clastogenic activity in serum from scleroderma patients was investigated by culturing lymphocytes from three normal individuals in medium enriched with serum from either a normal control, an ACA-negative scleroderma patient or an ACA-positive scleroderma patient. There was no statistically significant difference in the frequency of sporadic chromosomal abnormalities among the cells in these experiments. The results of this study suggest that, contrary to previously reported studies, the frequency of sporadic chromosome abnormalities is not increased significantly in scleroderma patients. In addition, although the anticentromere antibody is reactive with chromosomal material, patients with this antibody do not have increased chromosome breakage or aneuploidy, and the antibody does not induce chromosomal changes in vitro.
Adolescent↗
[A syndrome of facial hemiatrophy, circumscribed scleroderma and focal epileptic seizures associated with bilateral uveitis].
A peculiar form of sclerodermia is described in a 10 year girl. Initially, the disease pronounced itself with focal epileptic spells involving lated the focal sclerodermia and facial hemiatrophy. The peculiar feature of the case is the bilateral uveitis and subclinical serose meningitis. Brain CT scans and Doppler investigation of extracranial carotid and vertebral arteries were performed.
Child↗
Pulmonary arterial histology and morphometry in systemic sclerosis: a case-control autopsy study.
Morphometric measurements were performed on pulmonary arteries in 58 patients with systemic sclerosis (20 limited cutaneous and 38 diffuse cutaneous involvement [21 with and 17 without renal crisis]) and age, race, and sex matched autopsy controls. Matched pairs analysis was employed. For arteries of all sizes, the area of the intima and percent luminal occlusion were greater in the limited and diffuse (no renal crisis) groups than in controls, and these differences were statistically significant for large and medium sized vessels. The greatest luminal occlusion was found in limited cutaneous patients, and especially those with clinical evidence of pulmonary arterial hypertension, providing a rationale for the poor response to vasodilator therapy in these patients.
Constriction, Pathologic↗
[The different clinical variants of scleroderma in diabetics].
The course of scleroderma is compared in patients with and without diabetes mellitus. In combined pathology, bullous foci of injury and trophic abnormalities occur more frequently. All the patients were diagnosed to have abnormalities on the part of connective tissue metabolism.
Adolescent↗
[Generalized morphea-like progressive systemic sclerosis with anticentromere antibodies and eosinophilic cellulitis].
A 67-year-old woman without any history of exposure to organic solvents suffered from Raynaud's phenomenon, sclerodactylia, contracture of finger joints, diffuse pigmentation, pulmonary fibrosis, and generalized morphea-like eruptions on the trunk; she was diagnosed as generalized morphea-like progressive systemic sclerosis. She had a high titer of anticentromere antibody in her serum without any symptoms of CREST syndrome. She also had eosinophilic cellulitis on her extremities, which subsided within 6 months, and seemed to be due to a hypersensitivity reaction to mosquito bites. The occurrence of these two diseases together in our case may suggest some similarities in their pathogenesis.
Aged↗
[Scleroderma, an ageing process? I. Clinical and immunological aspects (author's transl)].
Scleroderma with its different manifestations is mainly a disease of connective tissue and of vascular system. Next the alteration to collagen, which is demonstrable in lesions by decrease of embryonale collagen type III, there are also humoral and cellular phenomens of autoimmunity. In more than 70% of our patients we found antinuclear antibodies, and in most of them, we found with the leukocytemigration--inhibition-test cellular immunphenomenons to RNA, collagen and muscle. There is a small connection between ageing and immunological defense and repair, accordingly of immunocytes and fibroblasts. Further more precise characterization of this cell-compartments under the aspect of a premature ageing could bring a new understanding in the largely unknown etiopathogenese of scleroderma.
Aging↗
The scleroderma neck sign.
The scleroderma neck sign, as described by Barnett, is a visible and palpable tight band over platysma in the hyperextended neck. A recent survey of 76 patients with scleroderma revealed that more than 90% had the scleroderma neck sign. Our study was performed using 15 patients with scleroderma and 30 controls including 3 with primary Raynaud's disease to examine the specificity of the scleroderma neck sign, and to look for a correlation between the presence of the scleroderma neck sign and histological changes of scleroderma in the skin overlying platysma. The scleroderma neck sign was present in 12 of the 15 patients with scleroderma but in none of the 30 controls. It was found both in patients with diffuse (5 out of 5) and limited (7 out of 10) scleroderma. In 10 of the 12 cases where the scleroderma neck sign was positive, there were characteristic histological changes of scleroderma on biopsy of the skin overlying platysma, in 1 there were nondiagnostic abnormalities, and in 1 the biopsy was unsatisfactory. The 3 patients with scleroderma in whom the scleroderma neck sign was absent had either nondiagnostic changes (1) or normal biopsies (2). The 3 patients with Raynaud's disease had normal skin biopsies. The scleroderma neck sign appears to be produced by scleroderma changes in the skin of the neck. In limited or early scleroderma where these changes are otherwise clinically inapparent, the scleroderma neck sign may be diagnostically useful.
Adult↗
[Scleroderma: oral manifestations].
Oral signs of sclerodermia and dental implications are described. The disease can be classified among the collagenopathies and its onset often involves dermatological signs prior to involving other organs and systems.
Humans↗
[Vasculitis associated with connectivitis].
A great number of vasculitis in childhood is associated with Rheumatic disease. Vascular lesions involve small vessels of various organs. These are described in systemic lupus erythematosus, juvenile rheumatoid arthritis, dermatomyositis, scleroderma, mixed connective tissue disease, Behçet syndrome, Sjögren syndrome.
Adolescent↗
[Scleroderma and thyroid diseases].
The authors report a prospective study of the thyroid function of 18 consecutive patients with systemic scleroderma and 7 patients with morphea. A history was taken and patients were examined for thyroid disease. The patients were tested for free thyroxin, TSH, cholesterolemia, circulating TeBG, anti-microsomial and anti-thyroglobulin antibodies and had a TRH test. The incidence of thyroid diseases was correlated with an age and sex-matched control population consisting of 2,534 subjects of the Loire department. None of the patients with morphea had a history of thyroid pathology and the thyroid screenings were within normal limits. A familial history of thyroid disease was found in 7 out of 18 patients with systemic scleroderma, and 8 out of 18 patients had a thyreopathy (one Hashimoto's disease, one Graves' disease, one toxic adenoma, one hypothyroidism, two euthyroid goiters, two nodular thyroids). The TRH test only revealed a toxic adenoma. A thyroid disease preceded systemic scleroderma in 5 out of 18 cases with delays ranging from 1 to 38 years. In 3 cases, the thyroid disease occurred within two years after the onset of systemic scleroderma. The prevalence of thyroid disease was significantly higher than in a comparable population sample of subjects from the same geographic region (p less than 0.01). The authors review the literature and discuss the physiopathologic relationships between the two clinical entities. The authors suggest to perform a thyroid screening (ultrasensitive TSH assay) on all patients with systemic sclerosis and especially on those whose clinical follow-up is atypical and on those with a familial and/or personal history of thyroid disease.
Aged↗