1/15. Scleroderma-like lesions in insulin-dependent diabetes mellitus.We report on two patients (one female 42 years, one male 47 years) suffering from insulin-dependent diabetes mellitus (IDDM) for more than 20 years. Both patients exhibited sclerodactyly and sclerosis of the hands and lower arms as well as swelling and slight contracture of the distal interphalangeal joints. Interestingly, internal organs were not involved and autoantibodies characteristic for scleroderma were missing. Poor utilization and excess of glucose seem to be responsible for the activation of fibroblasts to produce abundant matrix proteins in the skin. Significant therapeutic improvement of the glucose metabolism was able to improve joint contractures or at least to stop the progression of skin changes in our patients. These skin changes should not be misdiagnosed as systemic sclerosis.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
2/15. Treatment of generalized morphea with oral 1,25-dihydroxyvitamin D3.Scleroderma is a chronic connective tissue disease characterized by excessive collagen synthesis and its deposition in the skin and various internal organs. immune system abnormalities and disturbances of connective tissue metabolism have been suggested to play a central role in the pathogenesis of scleroderma. 1.25-Dihydroxyvitamin D3 (1.25(OH)2 D3 causes inhibition of fibroblast growth, has a role in controlling collagen synthesis and deposition and has numerous immunoregulatory activities. We assessed the effects of oral 1.25 (OH)2 D3 in the treatment of patients with generalized morphea. Three patients with generalized morphea, entered an open prospective study. They were treated with oral calcitriol (1.25 dyhidroxyvitamin D3) in an oral daily dose of 0.50-0.75 microgram. After the treatment period of 4-6 months, a significant clinical improvement was observed. The mobility of the joints improved, the skin extensibility increased and a substantial improvement of the skin induration. No serious side effects were observed. The improvement persisted after discontinuation of therapy during a follow-up period of one year. The evolution of the patients' condition during the 6 months therapy with calcitriol, suggests that it can be used as a beneficial agent in the treatment of generalized morphea. Double-blind, placebo-controlled trials are needed to assess its therapeutic value and a larger number of patients is desirable.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
3/15. Free tissue transfer in the treatment of linear scleroderma.Linear scleroderma is a subtype of localized scleroderma that most commonly affects children. The disease is rare, and the course is unpredictable. Most cases spontaneously resolve in 3-5 years. A small number, however, slowly progress to involve deeper subcutaneous tissues, muscles, and periosteum. Subsequent joint contractures and gross disturbances are frequent. Little on treatment is recorded in the literature. We report on four children who, in addition to bony procedures and release of joint and ligamentous contractures, had excision of the diseased skin and free soft-tissue transfer for coverage.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
4/15. Scleroderma: a case report of possible cause of restricted movement of the temporomandibular joint with effects on facial development.Scleroderma is a rare abnormality in which fibrosis of the skin, subcutaneous tissues and muscle may occur. All forms of scleroderma are rare in childhood: the most common form is localized scleroderma. Localized scleroderma is often benign, but may cause significant deformity, if it occurs on the face or extends across joint surfaces. Structural changes may occur in the osseous tissue and result in mandibular joint restriction (pseudoankylosis) and facial and occlusal disharmonies. This article describes a case of facial linear scleroderma in a nine-year-old child and the treatment performed to minimize consequences on occlusion and face development. Available clinical data will be illustrated.- - - - - - - - - - ranking = 3keywords = joint (Clic here for more details about this article) |
5/15. Tight skin and limited joint movements as early presentation of Hutchinson-Gilford progeria in a 7-week-old infant.We present a 7-week-old male infant with pseudoscleroderma as a primary manifestation of the Hutchinson-Gilford syndrome of premature aging. He had suffered intra-uterine growth retardation; micrognathism and a cleft palate were evident at birth. He presented with feeding difficulties and severe, diffuse scleroderma-like lesions, a faint peri-oral cyanosis and prominent scalp veins. With time, special facial features became more and more apparent: frontal bossing, prominent eyes, thin and fine nose and lips, microstomia, low-set ears and occipito-parietal alopecia. Histopathology of the skin showed an increased density and thickness of collagen in the dermis and hypodermis. Within the 1st year of life, typical skeletal characteristics were observed. The diagnosis of Hutchinson-Gilford syndrome was confirmed by analysis of the lamin A gene, revealing a heterozygous c.1824C > T (G608G) mutation. CONCLUSION: Hutchinson-Gilford syndrome is an extremely rare disorder of which the full clinical spectrum becomes evident with time. Sclerodermatous changes in the infant can be the first manifestation.- - - - - - - - - - ranking = 2keywords = joint (Clic here for more details about this article) |
6/15. Eosinophilic fascitis (Shulman syndrome) in a 13-year-old girl.Symptoms compatible with the diagnosis of eosinophilic fasciitis (morphea-like changes of the skin, restricted joint movement, eosinophilia, elevated immunoglobulin levels in serum) in a 13-year-old girl and the response to systemic corticosteroid treatment are reported. The literature is reviewed.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
7/15. Treatment of severe localized scleroderma by plasmapheresis--report of three cases.We report three patients with severe, localized scleroderma, and with elevated titres of antinuclear antibodies, who were treated by plasmapheresis in combination with systemic steroid therapy. The therapeutic effectiveness of plasmapheresis was assessed on the basis of improvement in cutaneous and joint lesions. In all cases, significant improvement occurred after 2 months of therapy. Thus, in addition to treating systemic sclerosis, plasmapheresis can also be recommended for treatment of severe cases of localized scleroderma with elevated titres of antinuclear antibodies and antibodies to ss-dna.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
8/15. Juvenile scleroderma: report of a case.Scleroderma is a rare connective tissue disease in children. A 12-year-old boy suffered from progressive increasing skin tension with erythematous changes in his left leg for a period of 3 months. This limited the range of motion in his left first and second metatarsophalangeal joints. A skin biopsy showed hypertrophic collagen bundles with atrophic skin appendages and lymphocytic infiltration. Based on the clinical manifestations and typical histopathologic findings, juvenile linear scleroderma was diagnosed. He was successfully treated with a short course of oral prednisolone in addition to long-term therapy with D-penicillamine and a topical emollient.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
9/15. Oral calcitriol as a new therapeutic modality for generalized morphea.BACKGROUND: None of the commonly used drugs for the treatment of scleroderma appears to significantly influence the fibrotic stage of this disorder. Recently, a beneficial effect of the treatment with oral calcitriol (1,25 dihydroxyvitamin D3) in 10 patients with systemic sclerosis and four patients with morphea was described. This fact could be ascribed to the immunoregulatory effects of calcitriol observed in vitro and to inhibition of fibroblast growth. We treated three patients with extensive morphea with remarkable results. observation: Three patients with generalized morphea were treated with calcitriol in an oral daily dose of 0.50 to 0.75 microgram. After 3 to 7 months of treatment, the mobility of the joints improved and the skin extensibility increased. No adverse effects were observed. The improvement persisted after discontinuation of therapy during a follow-up period of 1 to 2 years. CONCLUSION: calcitriol showed a beneficial effect in generalized morphea during an open study. Double-blind, placebo-controlled trials are needed to assess its therapeutic value.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
10/15. Localized scleroderma in childhood: a report of 30 cases.Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.- - - - - - - - - - ranking = 0.5keywords = joint (Clic here for more details about this article) |
| | Next -> |