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PubMedDate not supplied

[Spontaneous pneumoperitoneum in progressive systemic sclerosis (generalized scleroderma)].

In a twenty-nine year old female patient with progressive systemic sclerosis (scleroderma) (PSS) and an impressing involvement of the intestine (disturbance of the oesophageal motility, pseudo-obstruction, malabsorption) a spontaneous, asymptomatic pneumoperitoneum is observed. Perforation or pneumatosis cystoides intestinalis (PCI) could not be proved. The possible pathogenesis, course, prognosis and therapy of this rare complication are discussed.

Adult↗
PubMedDate not supplied

[Digestive symptoms of scleroderma (author's transl)].

The authors successively study the oral, peri-oral, esophageal, intestinal and anal manifestations of systemic scleroderma. They point out that dental, salivary, and esophageal involvement is of great interest for diagnosis and prognosis. They particularly describe the malabsorption syndrome including anatomical and ultrastructural lesions of the smooth muscle fibers. They present an original study of arrector pili in clinically and histologically non-involved skin of patients undergoing scleroderma. Finally they state that any suspected or diagnosed scleroderma needs a digestive check-up as follows: radiologic examination of the teeth, biopsy of the minor salivary glands of the lower lip, radiologic study of esophageal motility and radioactive C 14 glycocholate test.

Esophageal Diseases↗
PubMed2004

Systemic inflammatory diseases and silicone breast prostheses: report of a case of adult still disease and review of the literature.

There has been a debate about the possibility of a link between silicone breast implants and the onset of systemic connective tissue diseases (eg, scleroderma, systemic lupus erythematosus, rheumatoid arthritis) and other inflammatory pathologies, such as silicone implant associated syndrome and adult Still disease. We report a case of adult Still disease in a patient with a silicone gel breast implant. The disease regressed with steroidal treatment, and the patient is now no longer steroid-dependent, although the implant is still in place.

Adult↗
PubMed1984

Autoimmune disease developing after augmentation mammoplasty: report of 3 cases.

Three patients who developed autoimmune disease after mammoplasty are described. One had features similar to systemic scleroderma, one was similar to systemic lupus erythematosus whilst the 3rd has features of idiopathic thrombocytopenic purpura. All 3 patients had augmentation mammoplasty 5-25 years prior to development of autoimmune disease. A possible causal relationship between mammoplasty and the development of autoimmune disease is discussed.

Autoimmune Diseases↗
PubMed1989

[Scleroderma and HLA antigens].

A possible HLA disease association was investigated in 40 patients (38 female, 2 male) with progressive systemic scleroderma (PSS), and 42 patients (32 female, 10 male) with morphea. HLA ABCDR/DQ, glyoxalase and properdin factor B (GLO and BF) phenotypes of patients were compared with 193 healthy controls. Four PSS family studies were performed. The following relative risk (rR) values were determined in PSS: A1 (1.38), A2 (1.39), B8 (1.67), B15 (3.22) and in morphea: A3 (1.43), B7 (1.39), B40 (1.81), BW60 (2.49), DR2 (2.38) and DRW8 (2.55), indicating a relatively weak, HLA-linked genetic predisposition for the manifestation of these dermatological disorders. The HLA "risk" antigens for the two clinically different subtypes of the disease are also different: raised A1/B8 frequencies such as those in our PSS group are related to high (or pathologic) immune response (autoimmune disorders). In contrast, A3 B7 DR2 elevations such as those recorded in our morphea group correlate with low immune response. Following exposition to certain suspected environmental factors (quartz, chemical solvents, drugs, viral fragments), the HLA phenotype may thus predipose some individuals--predominantly women--to different clinical patterns of the disease. HLA typing may thus be useful in clinical differential diagnosis (recent subtyping protocols) and possibly also for determination of the prognosis, i.e. HLA-B8 seems to be related to an acute, inflammatory course of PSS, and HLA-B7/DR2, to rather mild morphea patterns.

Adult↗
PubMed2004

Drug eruption caused by azathioprine: value of using the drug-induced lymphocytes stimulation test for diagnosis.

Azathioprine (AZA) is an immunosuppressant commonly used for organ transplantation and autoimmune diseases. Allergic side effects of AZA are rare, and reported allergic skin eruptions from AZA are very limited in Japan. We report AZA-induced drug eruption that developed in two cases of systemic scleroderma with polymyositis. One case presented with Stevens-Johnson syndrome, and the other had systemic papular erythema. The stimulation indices of the drug-induced lymphocyte stimulation test (DLST) for AZA in these two patients were as high as 2,180% and 430%, respectively, but those of healthy volunteers were under 120% without nonspecific suppression of lymphocyte proliferation. Other drugs used simultaneously were ruled out by patch and challenge tests. The challenge test for Stevens-Johnson syndrome type drug allergy is very risky. DLST is a good diagnostic tool for AZA allergy, especially for severe drug allergy cases.

Azathioprine↗
PubMed2003

[Eosinophilic fasciitis--current knowledge].

First time eosinophilic fasciitis (EF) was described by Shulman. It occurrences predominantly in young men, rarely older women, sporadic in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophil count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous induration. The histopathologic examination in the region of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinphils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified to wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.

Autoimmune Diseases↗
PubMed2004

[Eosinophilic fasciitis--current database].

For the first time eosinophilic fasciitis (EF) was described by Shulman. It occurs predominantly in young men, rarely in older women and sporadically in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophils count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous indurations. The histopathologic examination of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinophils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified into wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.

Diagnosis, Differential↗
PubMed1993

[Peri-ungual capillaroscopy. Value in the diagnosis of systemic diseases].

The diagnostic value of nailfold capillaroscopy was assessed through blind analysis of a continuous series of 354 examinations. Major and minor dystrophies and capillary bed abnormalities are the best criteria, especially in patients presenting with vascular disorders of the upper extremities. A systemic disease (specificity: 82.7 percent; negative predictive value (NPV): 90.2 percent) and specifically a systemic scleroderma (sensitivity: 97.1 percent and NPV: 99.4 percent) is improbable in case of normal capillaroscopy. First component analysis distinguished patients with definite systemic disease from normal subjects. Its value is dubious in other connective tissue diseases systemic lupus erythematosus, rheumatoid arthritis, Sjögren's disease, etc.).

Adult↗
PubMed1992

Increased adhesion of fibroblasts from patients with scleroderma to extracellular matrix components: in vitro modulation by IFN-gamma but not by TGF-beta.

A characteristic feature of systemic scleroderma is fibrosis of the skin and eventually of internal organs resulting from an overproduction of collagen and other connective tissue components by the resident fibroblasts. The balance between the cells and the amount of the surrounding extracellular matrix is then altered. Because cellular metabolism depends to a large extent on cellular contacts and communications with connective tissue molecules, we have therefore investigated the interactions with extracellular matrix components of fibroblasts obtained from skin of patients affected with scleroderma. In comparison to fibroblasts from healthy skin, all fibroblasts from scleroderma patients had an increased adhesion capacity to collagens I, IV, VI, fibronectin, and laminin. In addition, whereas adhesion of control fibroblasts was stimulated by a pre-treatment with transforming growth factor-beta, adhesion patterns of scleroderma fibroblasts remained unchanged. However, pre-incubation of the cells with interferon-gamma decreased the adhesion of both scleroderma and control fibroblasts.

Cell Adhesion↗
PubMed1985

[Connective tissue compartments in scleroderma. Study of the structure and the biomechanical properties].

A combined histological and biomechanical study was performed in patients with localised or systemic scleroderma. The inflammatory reaction was variable in intensity and location, predominating around the superficial vascular plexus, the deep dermo-hypodermal plexus, the paraseptal plexus or in the fasciae. Secondary sclerosis resulted from the lateral fusion of bundles of collagen fibers. Three degrees of intensity of sclerosis were disclosed by our noninvasive technique of tonometry by vertical traction of the skin in vivo. That measurement allowed a follow-up of the evolution of the disease and a control of the therapeutic efficacy.

Connective Tissue↗
PubMed1991

Human basophil releasability. VIII. Increased basophil releasability in patients with scleroderma.

We evaluated basophil releasability in 16 female patients with scleroderma (systemic sclerosis) and in 16 normal age- and sex-matched donors. Basophils from patients with scleroderma released significantly more histamine "spontaneously" than did those from normal donors (12.9 +/- 2.1% versus 4.5 +/- 0.7%; P less than 0.0005). Basophil reactivity (maximal percentage histamine release) to anti-IgE was higher in patients with scleroderma than in controls (57.0 +/- 7.5% versus 35.4 +/- 7.8%; P less than 0.05). Basophil sensitivity (the concentration of anti-IgE that causes 40% of maximal percentage histamine release) to anti-IgE in scleroderma patients was similar to that found in controls (4.6 +/- 2.8 x 10(-2) micrograms/ml versus 2.3 +/- 1.0 x 10(-1) micrograms/ml; P not significant). Scleroderma patients also showed enhanced releasability compared with that of the controls when challenged in vitro with interleukin-3 (8.3 +/- 1.7% versus 3.2 +/- 0.6%; P less than 0.01). Releasability induced by the formyl-containing tripeptide, f-met peptide, was significantly higher in the scleroderma patients than in the controls at the 2 lower concentrations used. No differences in basophil reactivity and sensitivity to f-met peptide and calcium ionophore A23187 were found between patients and normal donors. These results show that spontaneous basophil releasability and releasability in response to IgE cross-linking and activation of interleukin-3 receptors are increased in patients with scleroderma.

Adult↗
PubMed1988

Biochemical characterization of scleroderma-inducing glycosaminoglycan.

The scleroderma-inducing N-sulfated glycosaminoglycan previously isolated by us from the urine of patients with systemic scleroderma was further purified: it was biochemically characterized by low O-sulfation and relative high N-sulfation. Consistent with this finding, desulfated and N-resulfated heparin, which had a similar composition to the urine-derived scleroderma-inducing glycosaminoglycan, induced a significant degree of sclerotic fibrosis in the skin of mice which had received intraperitoneal injections of it, whereas N-desulfated heparin with contrasting sulfation to it failed to cause any significant change in the skin.

Animals↗
PubMed1977

Cellular infiltrates in scleroderma skin.

The purpose of this study was to determine the frequency, distribution, and nature of cellular infiltrates in 108 skin biopsies from patients with systemic scleroderma (SS) and localized scleroderma (LS). Cellular infiltrates, perivascular or diffuse, were noted in 49% of SS and 84% of LS patients and consisted of lymphocytes, plasma cells, and macrophages. No correlation was noted between the presence or severity of skin cellular infiltrates and serum serologic abnormalities.

Adolescent↗
PubMed1995

Antihistone antibodies in scleroderma.

BACKGROUND: Antihistone antibodies (AHA) are usually considered the serological marker of drug-induced lupus erythematosus, but recently they have been found in patients with systemic scleroderma (SSc) and morphea. OBJECTIVE AND METHODS: We determined AHA in 43 patients with SSc, 4 patients with overlap syndrome and 11 with morphea. RESULTS AND CONCLUSIONS: AHA were demonstrated in 41.8% of the SSc patients and in 36.3% of the morphea patients. Only 1 patient with overlap syndrome had AHA. Our SSc patients with AHA had frequent cardiac and renal involvements suggesting a prognostic value of AHA. In our morphea patients AHA did not correlate with clinical features.

Adult↗
PubMed1987

Scleroderma of the breast: light and electron microscopy study.

The initial presentation of scleroderma (systemic sclerosis) with a manifestation involving only the breast has not been reported in the literature. Previous reports document scleroderma occurring after cosmetic mammaplasties. The presumed etiology is related to silicone, which has some adjuvant effect in development of the disease. This report documents a case of scleroderma presenting as a breast problem in a 28-year-old woman without prior exposure to silicone. We also describe the light and electron microscopy findings.

Adult↗
PubMed1995

Position 26 of the first domain of the HLA-DQB1 allele in post-silicone implant scleroderma.

OBJECTIVE: To determine whether women with scleroderma (systemic sclerosis, SSc) and silicone implants have the same or distinctive immunogenetic findings compared to those reported for idiopathic scleroderma. METHODS: In this case-control study, 9 Caucasian women with SSc and silicone implants (7 breast, 1 chin, 1 toe) and 128 healthy Caucasian controls were typed for HLA class II (DRB1,3,4,5, and DQB1) by DNA polymerase chain reaction (PCR) sequence specific oligonucleotide probes (SSOP). RESULTS: All women with SSc had HLA-DQ5 or DQ7 (DQB1*0301). These 2 alleles have glycine (Gly) or tyrosine (Tyr), and not hydrophobic leucine (Leu), at position 26 in the 2nd hypervariable region of the DQB1 first domain. The increased frequency of at least one Leu 26 negative allele (Gly + or Tyr +) in the women with SSc (100%) compared with controls (73%) was not statistically significant. In contrast, the low frequency of one Leu 26+ allele in the patients (28 vs 57%, p = 0.03, RR = -3.3) and 2 Leu 26+ alleles (0 vs 35%, p = 0.03, RR = -10.4) was significant. CONCLUSION: The presence of Gly 26 or Tyr 26 in the HLA-DQB1 first domain in our cases with SSc and silicone implants is consistent with immunogenetic findings reported in Caucasian with idiopathic SSc anticentromere autoantibodies. Whether all the immunogenetic features in SSc associated with silicone implants remain indistinguishable from those seen in idiopathic.

Alleles↗