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PubMedDate not supplied

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.

Ankle Joint↗
PubMed1986

Toxic oil syndrome: a syndrome with features overlapping those of various forms of scleroderma.

Thirty-two toxic oil syndrome (TOS) patients were selected because they presented with scleroderma-like changes and were observed during the first 36 months of evolution of the disease. Initially, these patients presented with a noncardiogenic pulmonary edema, eosinophilia, arthralgia/arthritis, peripheral edema, and myositis. Histologic investigations showed a widespread chronic interstitial infiltrate with lymphocytic vasculitis. They subsequently developed peripheral neuropathy, joint contractures, scleroderma-like changes, Raynaud phenomenon, pulmonary hypertension, sicca syndrome, and liver disease. Biopsy studies during this stage showed fibrosis and obliterating arteriopathy. Late features of TOS are musculoskeletal pain, cramps, livedo reticularis, carpal tunnel syndrome, and digital tuft changes. TOS is a new chemically induced scleroderma-like syndrome with features overlapping those of eosinophilic fasciitis, systemic sclerosis, and forms of localized scleroderma.

Adolescent↗
PubMed2003

Life with a rare chronic disease: the scleroderma experience.

BACKGROUND: Little is known about the experience of living with a rare disease and how people with rare diseases cope with not only the disease but also the reactions of others. Scleroderma is a rare chronic connective tissue disease that results in fibrotic changes involving all or some organs of the body. The two types of scleroderma are systemic scleroderma, which involves the skin and internal organs and is the more serious type, and local scleroderma, which attacks the skin and surrounding tissues. Some people with scleroderma have signs that are visible to outsiders, while others have invisible signs. Having this chronic condition and being different from the general population may subject people with scleroderma to stigmatization by others. AIM: The aim of this study was to understand, from the individual's perspective, the experience of living with scleroderma. METHOD: Focus group interviews were conducted with two groups of individuals with scleroderma. Because of the rarity of the disease and the illness of the participants, only two groups were held. The same questions were asked of both groups. A moderator and assistant guided the groups. FINDINGS: Data analysis revealed five themes: physical manifestations, disclosure/non-disclosure to others, living, being normal and facing the future. The data are discussed in light of participants' having visible signs, invisible signs and the rarity of their condition. For those with visible signs, disclosure was automatic. They were conscious of being different from others without scleroderma. Those with invisible signs managed their disease information in such a way as to minimize the stigma of being different. The rarity of the disease added the problem of others not understanding their difficulties. Those who disclosed their disease not only had to deal with the reactions of others, but faced the additional burden of having to explain their condition. CONCLUSION: Nurses may have little knowledge about scleroderma. It is possible that they, through their ignorance of such rare conditions, may stigmatize individuals. Through understanding about rare diseases will they be able to teach patients the skills necessary to help them cope with their symptoms, as well as the reactions of others to their diagnosis and appearance.

Adaptation, Psychological↗
PubMed2001

Localized and systemic scleroderma show different histological responses to methotrexate therapy.

Although morphoea (localized scleroderma) and systemic sclerosis are distinct disease entities, the skin lesions show identical histological characteristics and both diseases respond favourably to low-dose treatment with methotrexate (MTX). The aim of this study was to find out whether MTX treatment induces different histological response patterns in these two diseases. In seven patients with morphoea and eight with systemic sclerosis, skin biopsies were taken before and after 24 weeks of treatment with low-dose MTX. In the centre and active margin of morphoea lesions, a significant reduction in tenascin staining was seen after 24 weeks of treatment, in contrast to systemic sclerosis. The numbers of mast cells decreased in the active margin of morphoea lesions, whereas in systemic sclerosis no significant change was seen after MTX therapy. Epidermal proliferation and staining of heparan sulphate proteoglycans showed no changes. Although skin lesions from both diseases respond clinically to treatment with MTX, systemic sclerosis shows no change in the immunohistochemical parameters investigated, whereas morphoea does. This difference in dynamic pattern suggests that the apparently similar lesions in localized and systemic sclerosis are not identical.

Adult↗
PubMed1996

Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma.

The mechanism that may cause degenerative fibrotic skin lesions was studied in situ using skin biopsies from patients with systemic sclerosis (SSc), localized scleroderma, or keloids, and at the initial disease stage in the University of California at Davis (UCD) lines 200/206 chickens, which develop a hereditary systemic connective tissue disease resembling human SSc and permit study of disease stages not accessible in humans. Frozen skin sections were analyzed simultaneously for apoptosis by terminal deoxynucleotidyl transferase-mediated FITC-dUTP nick end labeling and indirect immunofluorescence staining of cell markers with tetramethylrhodamine isothiocyanate conjugates. The results showed that endothelial cells are clearly the first cells to undergo apoptosis in the skin of UCD-200/206 chickens, a process that seems to be induced by anti-endothelial cell antibodies. In human fibrotic skin diseases, apoptotic endothelial cells could only be detected in early inflammatory disease stages of SSc and localized scleroderma.

Adolescent↗
PubMed1998

Dermal mast cells in scleroderma: their skin density, tryptase/chymase phenotypes and degranulation.

To determine the distribution, tryptase/chymase phenotypes and degranulation of mast cells (MCs) in the dermis of patients with scleroderma, we examined MC density in the skin of 22 patients with systemic sclerosis (SSc) and 11 with localized scleroderma (LSc). We used antitryptase and antichymase antibodies after Carnoy's fixation. Detailed reports of two representative patients with SSc and LSc are included. In the scleroedematous stage (grade 1) showing oedema in both papillary and reticular dermis with variable homogenization of collagen bundles in the reticular dermis, MC skin density was variable in each specimen although MC skin density, as a whole, was significantly increased as compared with normal skin (P < 0.05). In the sclerotic stage (grade 2) characterized by homogenization of collagen bundles in the entire dermis, MC skin density was significantly decreased as compared with normal skin (P < 0.005). LSc showed changes similar to those in SSc. The ratio of MCTC cells (both tryptase- and chymase-positive MC) to MCT cells (tryptase-positive but chymase-negative MC) was variable in SSc and LSc. MCT cells were exclusively dominant in three patients with SSc and two with LSc. In a patient with SSc (patient 1) showing remarkable perivascular and interstitial oedema in the upper dermis, MC skin density was increased in the oedematous portion and tryptase-positive granules were distributed in extracellular locations. In another patient with LSc (patient 2), tryptase positivity increased and chymase positivity decreased in both number and intensity as the skin sclerosis progressed. MCs must have variable interactions with the lesional skin in SSc and LSc. The present study suggests that MCs are involved in the development of interstitial oedema.

Adult↗
PubMedDate not supplied

[Physical factors in the pathogenetic therapy of children with scleroderma].

Telecapillaroscopy, biomicroscopy of the bulbar conjunctiva, rheovasography, cold vascular test, ohyprolin and glycosaminoglycans excretion measurements have been performed in 337 2-14-year-olds with systemic and localized scleroderma to study peripheral circulation and connective tissue metabolism. The results contributed to development of differentiated balneo- and pelotherapy schedules affecting the principal components of the disease pathogenesis.

Adolescent↗
PubMed2002

A case of linear scleroderma with muscle calcification.

We report a 21-year-old man with linear scleroderma with muscle calcification revealed by a computed tomographic (CT) scan. Levels of serum calcium and phosphorus were normal. This is the first report of localized scleroderma with muscle calcification. Muscle involvement may cause contracture or functional abnormalities of the extremities. Therefore, CT scans may be useful in evaluating muscle involvement in patients with localized scleroderma.

Biopsy↗
PubMed2005

Sclerodermic linear lupus panniculitis: report of two cases.

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.

Adult↗
PubMed2005

Linear scleroderma en coup de sabre with associated neurologic abnormalities.

Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease.

Bell Palsy↗
PubMed1999

Scleroderma overlap syndromes.

The most common scleroderma overlap syndromes are mixed connective tissue disease (MCTD), scleromyositis and synthetase syndrome. There is controversy concerning MCTD as a separate entity due to heterogeneous clinical manifestations, not infrequent transformation into definite CTD and various classification criteria. Our study of 94 adult patients and 20 children, classified according to the criteria of Alarcon-Segovia, and especially a 5, 9-year follow-up showed transformation into SLE or SSc in over 20% of patients, less frequently than reported by others, whereas over half of the cases remained undifferentiated CTD. In several cases ARA criteria for both SSc and SLE were fulfilled, and there is no consensus whether such cases should be recognized as coexistence of both definite diseases or as MCTD. High titers of U1 RNP antibodies to 70 kD epitope were invariably present, whereas, by transformation into distinctive CTD there appeared, in addition, antibodies characteristic of these CTD. Of 108 cases positive for PM-Scl antibody, 83% were associated with scleromyositis. This scleroderma overlap syndrome differed from MCTD by coexistent features of dermatomyositis (myalgia, myositis, Gottron sign, heliotrope rash, calcinosis) with no component of SLE, characteristic of MCTD. The course was also chronic and rather benign, as in MCTD, and all cases responded to low or moderate doses of corticosteroids. A not infrequent complication was deforming arthritis of the hands. Our immunogenetic study showed an association of cases positive for PM-Scl antibody with HLA-DQA1x0501 alleles in 100% and with HLA-DRB1x0301 in 94% of cases. Synthetase syndrome, associated with anti-histidyl-tRNA synthetase antibodies, studied in 29 patients with myositis and interstitial lung disease (ILD), only in single cases had scleroderma-like features. These cases differed from SSc by acute onset with fever, and by response to moderate doses of corticosteroids. We also studied overlap of localized scleroderma with other CTD: 21 cases of progressive facial hemiatrophy and linear scleroderma, and 55 (39.5%) of atrophoderma Pasini-Pierini (APP) and morphea. As in other autoimmune disorders, two or more connective tissue diseases (CTD) may develop concurrently or sequentially in the same patient. In such overlap syndromes ARA criteria must be fulfilled for each of the disease, and the clinical presentation has features of both. However more frequently overlap syndromes only combine some manifestations of more than one CTD, and present a highly heterogeneous group of disorders with prevailing clinical features of SSc.

Adolescent↗
PubMed2000

Differential expression of nitric oxide by dermal microvascular endothelial cells from patients with scleroderma.

Vascular abnormalities in scleroderma are fundamental to the pathogenesis of this disease. The objective of this study was to characterize dermal microvascular endothelial cells (DMEC) isolated from scleroderma patients with respect to growth and expression of the constitutive form of endothelial nitric oxide synthase (eNOS). DMEC from patients with both systemic sclerosis (SSc) and localized scleroderma (Loc Scl) contained small intact microvascular structures in contrast to single cell isolations obtained from control skin. Immunoaffinity selection on anti-PECAM-1 beads yielded pure populations of DMEC expressing normal markers. While the morphology and initial growth of SSc DMEC closely paralleled control cells, the growth of SSc DMEC decreased with time in culture (doubling time of 3 days vs. 5 days). Expression of ecNOS mRNA was reduced in both Loc Scl and SSc as shown by semi-quantitative RT-PCR (p < 0.001). Western blots showed variable but generally lower ecNOS protein levels and decreased levels of nitrogen oxides in media were found from both SSc and Loc Scl relative to control cells. The results indicate an intrinsic defect in the mechanism of nitric oxide production in DMEC isolated from scleroderma patients and suggest its possible involvement in the pathophysiology of scleroderma.

Adult↗
PubMed2005

[Linear scleroderma and foot deformity in children].

Linear scleroderma is a subtype of localized scleroderma generally observed in children, and may produce secondary bone and joint deformities. Its localization at the foot or ankle is rarely reported. A complete review of the literature reveals 21 cases of foot or ankle deformity due to linear scleroderma to which we add one case. The clinical and radiological aspects of the deformity, its pathogenesis, histological findings as well as orthopaedic management are presented and discussed.

Child↗
PubMed1989

Antibodies to the Borrelia burgdorferi flagellum in patients with scleroderma, granuloma annulare and porphyria cutanea tarda.

It is generally accepted that cutaneous Lyme borreliosis comprises erythema chronicum migrans, lymphadenosis benigna cutis, and acrodermatitis chronica atrophicans. In recent years the tick-borne spirochete Borrelia burgdorferi has been associated with a number of other cutaneous disorders. We therefore investigated sera from 175 patients with localized scleroderma (morphea) (n = 64), systemic sclerosis (n = 74), granuloma annulare (n = 16) and porphyria cutanea tarda (n = 21) with the new, highly sensitive and specific Borrelia burgdorferi flagellum ELISA assay. As controls (n = 297) served normal healthy volunteers and patients with other skin diseases. It was found that the distribution of individual antibody values and the median antibody levels were identical in controls and in patients with scleroderma, granuloma annulare and porphyria cutanea tarda. These data do not support the hypothesis of an etiological association between Borrelia burgdorferi infection and scleroderma, granuloma annulare or porphyria cutanea tarda.

Adolescent↗
PubMed1992

In situ localization of interleukin-6 in normal skin and atrophic cutaneous disease.

Interleukin-6 (IL-6) is a multifunctional cytokine that participates in the inflammatory and immune responses. In human skin, keratinocytes produces IL-6, although the in vivo role of this cytokine is unknown. In the present study we investigated the in situ localization of IL-6 in normal epidermis (n = 10) and in a group of skin diseases characterized by epidermal atrophy. Formalin-fixed paraffin-embedded skin biopsies from patients with clinical and histopathological features consistent with localized scleroderma (n = 10), systemic scleroderma (n = 5), lichen sclerosus et atrophicus (n = 9) and balanitis xerotica obliterans (n = 7) were tested using polyclonal antibodies and avidin-biotin-peroxidase immunostaining. We demonstrated the presence of IL-6 in normal epidermis and in atrophic skin diseases. In normal skin there was moderate intercellular and intracellular reactivity detected using a high antibody concentration. In specimens with epidermal atrophy we detected intense cytoplasmic and intercellular immunostaining using a lower antibody concentration. The immunoreactivity was independent of the epidermal thickness. Plasma IL-6, measured by radioimmunoassay, was not elevated in plasma from patients with localized or systemic scleroderma. Increased IL-6 in the epidermis of selected skin diseases suggests that IL-6 may be related to the pathophysiology of dermatologic diseases characterized by epidermal atrophy.

Antibody Specificity↗
PubMed1986

Hyaluronate in suction blisters from patients with scleroderma and various skin disorders.

With a specific radioassay hyaluronic acid (hyaluronate) concentrations have been determined in suction blister fluid from abdominal skin and serum. Healthy subjects, patients with acrosclerosis, CRST, mucinosis and urticaria had 0.8-5.6 micrograms/ml of hyaluronate in their suction blisters, which is about 100 times more than the serum level. Increased concentrations were noted in blister fluid from patients with active lesions of systemic and localized scleroderma as well as lichen sclerosus. Here the increase could be due to an increased production of hyaluronate in the dermis. High levels of hyaluronate were, however, also found in blister fluid from patients with other types of inflamed skin. An increased leakage into the blister of hyaluronate from the dermis, probably through the lymph vessels, therefore seems best to explain the high concentrations of hyaluronate.

Blister↗
PubMed1983

[Significance of anti-centromere antibodies. Clinical value].

The authors report 38 cases of patients with antinuclear antibodies directed against the centromere of the chromosomes. These were 32 cases of scleroderma, 2 cases of Raynaud's syndrome, 2 cases of rheumatoid arthritis, 1 case of polymyositis and 1 case of disseminated lupus erythematosus. In the cases of scleroderma, anti-centromere antibodies were detected in 54 p. cent of cases of Crest syndrome, for which they are a good laboratory marker; they were found more rarely in case of sclerodactyly (26 p. cent of cases) and acrosclerosis (14 p. cent of cases) and not at all in cases of localized scleroderma, diffuse scleroderma or eosinophilic fasciitis. Anti-centromere antibodies may be the only antinuclear antibodies in these patients. They can be detected using rapidly dividing cell cultures.

Adult↗
PubMed2000

Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease.

OBJECTIVE: To investigate esophageal dysmotility and gastroesophageal reflux, which are important, but often occult features of gastrointestinal (GI) involvement in children and adolescents with mixed connective tissue disease (MCTD) and scleroderma. METHODS: We carried out intraesophageal 24 h pH monitoring of 14 patients with scleroderma and MCTD. RESULTS: We observed an elevated reflux index in 64% of the patients. An increased number of reflux events was found in 85%. Reflux events > 5 min were noted in 50% of the patients, which is indicative of possible development of esophagitis. Only 3 patients presented clinical symptoms. Four of 5 patients with localized scleroderma revealed a pathological reflux index. We found no relationship between gastroesophageal reflux, age of patients, duration of disease, and Raynaud's phenomenon. CONCLUSION: GI involvement in children with scleroderma or MCTD is more frequent than clinical symptoms indicate. An active diagnostic investigation for GI dysmotility and gastroesophageal reflux is necessary to detect these complications.

Adolescent↗