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PubMed2004

Aspects of nailfold capillaroscopy in children and young people with rheumatic disease.

Nailfold capillaroscopy was performed in children and young people with rheumatic disease (42 patients with a variety of childhood rheumatic diseases) and 30 normal control subjects. Distinctive morphologic abnormalities with capillary enlargement, megacapillary and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis (DM) and with scleroderma (SD). Capillary abnormalities were found in all 4 patients affected by systemic disease and in none of 7 patients with localized scleroderma. Some minor atypical capillary morphology were observed in normal individuals (37%) but the enlarged capillaries, especially megacapillaries were not found.

Adolescent↗
PubMedDate not supplied

Localized and systemic forms of scleroderma in adults and children .

Systemic sclerosis (SSc) presents a great deal of variability in the extent and severity of skin and internal organ involvement. The diagnostic and prognostic significance of autoantibodies in SSc is undisputed and the patient's autoantibody profile represents a fundamental tool for clinicians. Scleroderma is a rare condition in children. Unlike adults, localized scleroderma is more frequent than the systemic sclerosis, nevertheless it represents a disabling condition. In both conditions, no validated outcome measures and proven effective treatment is available to date.Raynaud's phenomenon (RP) is one the most common and significant clinical symptoms of SSc and therefore in patients with RP a capillaroscopic analysis should be carried out as soon as possible. The actual and select advantage of the early nailfold videocapillaroscopic (NVC) analysis is to distinguish between the primary RP and the secondary RP and to allow the early detection of SSc.

Adult↗
PubMed2005

Uterine prolapse in a young nulligravida with scleroderma and premature ovarian failure.

We report a case of pelvic organ prolapse quantification (POPQ) stage III uterine prolapse in a 25-year-old nulligravida. Premature ovarian failure was diagnosed after 1 year of amenorrhea. Localized scleroderma was noticed on her thigh and lower back. We discuss the possible role of scleroderma and ovarian failure on the occurrence of uterine prolapse in light of the literature.

Adult↗
PubMed1992

Increased expression of tenascin in the dermis in scleroderma.

The expression of tenascin, a recently discovered extracellular matrix protein, was studied by immunohistochemical techniques in scleroderma skin and compared with its distribution in normal skin. In progressive systemic sclerosis, a marked increase in tenascin content was observed in the superficial reticular dermis. In localized scleroderma, the deposition of tenascin was increased both in the superficial and deep dermis of involved skin, whereas in clinically uninvolved skin the distribution of tenascin was the same as in normal control skin, i.e. the papillary dermis and peri-appendiceal zone. The distribution of tenascin did not strictly parallel that of fibronectin. These findings and the current knowledge of tenascin biology suggest that the overproduction of tenascin in scleroderma dermis could be secondary to stimulation of fibroblasts by immune cell-derived cytokines, or could be due to abnormal fibroblasts, or a subpopulation of fibroblasts, producing high levels of this extracellular matrix protein.

Adult↗
PubMed1992

[Systemic scleroderma and scleroderma-like disease after silicone implants].

Systemic sclerosis or clinical patterns indistinguishable from systemic sclerosis have previously been demonstrated in workers in the polyvinylchloride industry and in mine-workers exposed to silica dust. In recent years, an increasing number of cases of systemic sclerosis, localized scleroderma, and scleroderma-like disease have been diagnosed in women after implantation of silicone gel prosthesis either following operations for breast cancer or cosmetic augmentation mammoplasty. We present two cases of systemic sclerosis or scleroderma-like disease appearing after silicone implants. Together with the already reported cases these results should lead to reduced use of silicone for cosmetic augmentation mammoplasty and a search for another material for patients operated on for breast cancer. The cases described, together with the reports concerning industrially provoked scleroderma, are of interest for the continued efforts to clarify the pathogenesis of scleroderma. Other types of exposure than those described here may also be of interest, i.e. occupational exposure to silicone spray.

Female↗
PubMed2003

Oral calcitriol: a new therapeutic agent in cutaneous lichen sclerosis.

BACKGROUND: Lichen sclerosus remains an elusive disease with an uncertain relationship to morphea and scleroderma. The disorder has been difficult to treat, with no consistent and reproducible efficacious therapy. Recently, a beneficial effect of treatment with oral calcitriol (1-25 dihydroxyvitamin D3) in patients with scleroderma or morphea was described. This fact could be ascribed to the immunomodulatory effects of calcitriol observed in vitro and to inhibition of fibroblastic growth. Because of the success of calcitriol in localized scleroderma, we attempted this therapy in a patient with LS. OBSERVATION: One patient with cutaneous generalized LS resistant to different therapeutics was treated with calcitriol in an oral daily dose of 0.5 mcg. After 6 months of treatment, the skin extensibility increased, and the lesions improved. The improvement persisted after discontinuation of therapy during a follow-up period of one year. The only side effect was hypercalciuria, which resolved with dose reduction. CONCLUSION: Calcitriol has shown a beneficial effect in scleroderma and morphea during open studies. A case is reported of a patient with LS who had a dramatic response to calcitriol. Double blind, placebo-controlled trials are needed to assess the therapeutic value of calcitriol in patients with LS.

Administration, Oral↗
PubMed1994

Successful amplification of DNA specific for Finnish Borrelia burgdorferi isolates in erythema chronicum migrans but not in circumscribed scleroderma lesions.

Early diagnosis of Borrelia burgdorferi infection, hampered by the absence of detectable antibodies in most patients with erythema chronicum migrans is important to prevent late-stage neurologic, rheumatologic, and skin disorders. Furthermore, B. burgdorferi has been claimed to be the causative agent in localized scleroderma (morphea). We used PCR amplification to search for B. burgdorferi outer surface protein OspA-specific sequences in DNA obtained from lesional skin biopsies on Finnish patients with clinically suspect erythema chronicum migrans, lymphocytoma, morphea, or with diverse skin manifestations and persistent high antibodies to B. burgdorferi flagellar antigen. Seronegative patients with other skin lesions served as controls. The amplicons obtained with primers specific for B. burgdorferi type strain B31 ospA sequence did not hybridize to the corresponding probes, and thus the DNA amplified from a Finnish B. burgdorferi erythema chronicum migrans skin isolate was sequenced. This 98-nucleotide sequence of ospA (332-429) showed 11% to 14% nucleotide divergence compared with the North American type strain (B31), several European strains, and an East Siberian tick strain. The sequence was almost identical (99%) to a Swedish isolate from acrodermatitis chronica atrophicans. Using oligonucleotides specific for the Finnish strain, a positive polymerase chain reaction-based hybridization was obtained in six of seven untreated erythema chronicum migrans patients infected in Finland or in Estonia, and in the lymphocytoma patient. Only two of the erythema chronicum migrans patients had IgG or IgM antibodies to flagellin. However, all seven morphea lesions as well as the other lesions were polymerase chain reaction negative. Polymerase chain reaction-based hybridization of B. burgdorferi OspA gene from skin-derived DNA thus provides a sensitive and specific diagnostic tool. In conditions not unequivocally known to be caused by B. burgdorferi, like in morphea, this assay was negative. We also demonstrate that peri-Baltic B. burgdorferi isolates show homology in their OspA genes but differ from geographically more distant isolates.

Antibodies, Bacterial↗
PubMed1978

Scleroderma, eosinophilia, and diffuse fasciitis.

Skin induration without internal organ involvement, blood and tissue eosinophilla, and fascilitis are features of diffuse fascilitis. However, cellular infiltrates (lymphocytes, plasma cells, and eosinophils) may also be present in the dermis, fat, and muscle. Blood eosinophilia (mild and transient) and skin eosinophilia were observed in about 20% of patients with systemic and localized scleroderma.

Adult↗
PubMed1981

Eosinophilic fasciitis: report of 15 cases.

Clinical and histopathologic features of 15 cases of diffuse fasciitis with eosinophilia are reviewed. The patients experienced an inflammatory scleroderma-like illness without Raynaud's phenomenon or internal organ involvement. Polyarthritis and carpal tunnel syndrome were observed. The clinical courses demonstrated the potential for spontaneous remission, relapse, and late recurrence. Histopathologic features are discussed, as is their relationship to other forms of localized scleroderma. None of the 12 patients treated with corticosteroids has had complete resolution of the skin changes after a median follow-up of 10 months. Unusual associated diseases--acute myelomonocytic leukemia and an evolving myeloproliferative disorder--were observed in two patients.

Adult↗
PubMed1992

Serum adenosine deaminase activity in systemic sclerosis (scleroderma) and related disorders.

BACKGROUND: Adenosine deaminase (ADA) activity in serum is mainly derived from T lymphocytes. OBJECTIVE: Our purpose was to clarify the significance of ADA activity in systemic sclerosis (PSS) and related disorders. METHODS: ADA activity was determined with an enzymatic method in 34 patients with PSS, 4 with mixed connective tissue disease (MCTD), 6 with dermatomyositis (DM), 11 with localized scleroderma (LS), and 13 with other collagen diseases. RESULTS: Serum ADA activity was elevated over the mean (+2 standard deviations) of the control in 85% of the patients with PSS, all with MCTD, 83% of those with DM, and 82% of those with LS. The mean values in 10 PSS patients with anti-topoisomerase I antibodies, 14 patients with anti-RNP antibodies, 12 patients with anticentromere antibodies (ACAs), and 5 patients without antinuclear antibodies (ANAs) were 26.1, 24.9, 22.6, and 16.8 IU/L, respectively. In most cases, except for ACA-positive patients, serum ADA activity changed almost in parallel with ANA titers. CONCLUSION: These results support the notion that T cells are involved in the pathogenesis of PSS and related disorders.

Adenosine Deaminase↗
PubMed1989

["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications].

We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.

Atrophy↗
PubMed2005

In vivo cutaneous interferon-gamma gene delivery using novel dicationic (gemini) surfactant-plasmid complexes.

BACKGROUND: Localized scleroderma (morphea and linear scleroderma) is a connective tissue disease, accompanied by excessive proliferation and deposition of collagen within the skin, inflammation, vasculopathy and a deranged immune system. Interferon gamma (IFNgamma), an inhibitor of collagen synthesis and an immunomodulator, could be a potential therapeutic agent if it could be delivered into or expressed locally in affected skin in a non-invasive manner. In this study, the feasibility of topical delivery of the IFNgamma gene and expression of IFNgamma were investigated in mice. METHODS: Novel dicationic (gemini) surfactant (spacer length n=2-16; alkyl chain m=12 or 16)-DNA complexes were formulated and characterized by circular dichroism and atomic force microscopy to select gemini analogues with the highest transfection efficiency (TE). Transfection and cellular expression of IFNgamma from the bicistronic pGTmCMV.IFN-GFP plasmid were evaluated in PAM 212 keratinocyte culture by ELISA and fluorescence microscopy. Topical delivery of plasmid using liposomal and nanoemulsion systems, based on gemini surfactant 16-3-16, was evaluated in mice by IFNgamma expression analysis. RESULTS: In vitro TE was found to be dependent on the spacer length of the gemini surfactant, with the C3 spacer showing the highest activity (both 12-3-12 and 16-3-16). Both gemini cationic liposomes and gemini nanoemulsion (3x25 microg DNA/animal) produced significantly higher levels of IFNgamma in the skin (359.4 and 607.24 pg/cm2) compared to naked DNA (135.69 pg/cm2) or a liposomal Dc-chol formulation (82.15 pg/cm2). IFNgamma expression in the lymph nodes was higher in the animals treated with gemini liposomes (422.74 pg/animal) compared to the nanoemulsion formulation (131.27 pg/animal) or the Dc-chol formulation (82pg/animal). CONCLUSIONS: The feasibility of topical delivery of pGTmCMV.IFN-GFP plasmid in mice using gemini cationic surfactant based delivery systems was demonstrated. IFNgamma expression after treatment with gemini-DNA formulations in the skin was 3-5-fold higher compared to the treatment with naked DNA (p<0.05), and 4-6-fold higher than the Dc-chol-DNA complex, indicating a significant advance in topical DNA delivery across intact skin in vivo.

Animals↗
PubMed1991

[Experimental study of progressive facial hemiatrophy: effects of cervical sympathectomy in animals].

Progressive facial hemiatrophy (Romberg's syndrome) is of unknown cause and uncertain pathogenesis. The main pathogenetic hypotheses are: sympathetic system alterations, localized scleroderma, trigeminal changes, possibly of genetic origin. To test the hypothesis of sympathetic system alterations, we designed an experimental model with ablation of the superior cervical sympathetic ganglion in rabbits, cats and dogs. All the animals were operated upon when 30 days old and were examined monthly for 1 year. During this period localized alopecia, corneal ulceration, keratitis, strabismus, enophthalmos, ocular atrophy, hemifacial atrophy and slight bone atrophy on the side of the sympathectomy were observed. Thus, cervical sympathectomy reproduces in animals the principal clinical alterations of Romberg's syndrome. Our data suggest that the sympathetic system is involved in the pathogenesis of this syndrome.

Animals↗
PubMed1998

Autoantibodies to C-reactive protein (CRP) and other acute-phase proteins in systemic autoimmune diseases.

Autoantibodies to CRP were reported previously in patients suffering from toxic oil syndrome. This syndrome resembles autoimmune diseases such as systemic lupus erythematosus (SLE) or systemic scleroderma. We therefore examined the prevalence of antibodies to CRP and other acute-phase proteins in autoimmune diseases, including SLE, subacute cutaneous lupus erythematosus (SCLE), systemic scleroderma (SSc), and primary biliary cirrhosis (PBC), as well as in bone marrow transplantation-induced chronic graft-versus-host disease and eosinophilia-myalgia syndrome. IgG antibodies to CRP were found in 78% of SLE and in 30% of SCLE patients, while 16% of patients with PBC were positive. In up to 45% of patients with SSc predominantly IgG antibodies to ceruloplasmin were detectable. Lack of systemic involvement as in discoid lupus erythematosus and localized scleroderma (morphea) correlated with low or absent antibody formation. However, no correlation was found between anti-acute-phase protein antibodies with liver disease or other organ involvement. Adsorption studies revealed that non-native epitopes on the CRP molecule, termed modified CRP, are the main target of antibodies. Chronic inflammatory tissue injury in systemic autoimmune disease might increase the presentation of cryptic epitopes of CRP to the threshold required for T cell activation.

Acute-Phase Proteins↗
PubMed1985

Hematologic disease in scleroderma syndromes.

Review of 4 430 patients with a diagnosis of scleroderma, morphea, or eosinophilic fasciitis has revealed 16 patients with one of a variety of serious hematologic diseases. Although it is not possible to comment on the exact relationship between the dermatologic disease and the hematologic disorder, in seven patients there appeared to be a close temporal relationship between the onset of the two conditions. The development of a serious hematologic disease, especially a lymphoproliferative process, may rarely be seen not only in patients with eosinophilic fasciitis but also in those with systemic or localized scleroderma.

Adult↗
PubMed1979

Concurrent linear scleroderma and systemic lupus erythematosus: a report of two cases.

Two patients with linear scleroderma (en coup de sabre) developed systemic lupus erythematosus (SLE). This association has been well documented in only one previous case. The presence of high titer antibodies to ribonucleoprotein (RNP) initially led to the diagnosis of the mixed connective tissue disease. Development of more serious clinical involvement and antibodies to Sm (case 1) or native deoxyribonucleic acid (nDNA) (case 2) helped establish a diagnosis of SLE. Use of these studies in the differential diagnosis of systemic rheumatic diseases is disucssed briefly. The presence of anti-RNAP antibodies in patients with localized scleroderma may herald a more serious rheumatic disease.

Adult↗
PubMedDate not supplied

Photodynamic therapy in dermatology.

Currently, topical photodynamic therapy (PDT) has received approval for the treatment of dermato-oncologic conditions like actinic keratoses, Bowen's disease, in-situ squamous cell carcinoma and basal cell carcinoma in many countries all over the world. For many non-neoplastic dermatological diseases like localized scleroderma, acne vulgaris and viral warts a therapeutical benefit of PDT is evident, too. Unlike the formerly used, only systemically-applicable haematoporphyrin derivates, the recently developed topical photosensitizers 5-aminolevulinic acid (ALA) or its methyl ester (MAL) induce photosensitizing porphyrins. Moreover, the latter do not induce strong generalized cutaneous photosensitization. Due to the easy accessibility of skin to light activation, incoherent lamps or LED arrays are suitable for PDT. The production of reactive oxygen intermediates like singlet oxygen depends on the applied light dose as well as the concentration and localization of the photosensitizer in the diseased tissue. Either cytotoxic effects resulting in tumor destruction or immunomodulatory effects improving inflammatory skin conditions are induced. Treating superficial non-melanoma skin cancer, PDT has been shown to be highly efficient despite the low level of invasiveness. The excellent cosmetic results after treatment are beneficial, too.

Basal Cell Carcinoma↗
PubMed1999

Investigation of sialic acids and sialyltransferase activity in blood of patients with systemic scleroderma.

Systemic scleroderma (SSd) is a connective tissue disorder accompanied by generalized fibrosis. A disturbance of the synthesis and production of matrix glycoproteins, such as collagens, fibronectin, and proteoglycans, by connective tissue cells is typical for this disease. We previously demonstrated a decrease in the ganglioside content of cultured skin fibroblasts from patients with SSd. In this work the contents of sialoglycoproteins and sialoglycolipids in blood sera of patients with SSd were estimated. Simultaneously, the level of asialofetuin-sialyltransferase activity in blood plasma of three groups of patients--those with SSd, Raynaud's phenomenon, and with localized scleroderma--was investigated. CMP-5-acetamido-9-deoxy-9-fluoresceinylthioureidoneuraminic acid was used as a substrate for the enzyme assay. It was shown that the concentration of total sialic acid was increased and the concentration of lipid-bound sialic acid was slightly decreased in the blood sera of patients with SSd. A correlation between the lipid-bound sialic acid level and the severity of disease was observed; there was no correlation between severity of disease and total sialic acid. Sialyltransferase assay showed a decrease in the activity level in all three groups of patients. The greatest decrease (2-fold) of this activity was observed in patients with Raynaud's phenomenon. Our data suggest that in SSd and similar diseases the process of glycoconjugate sialylation is disturbed. These changes may considerably affect the mechanisms of regulation of metabolism and cellular interactions.

Cells, Cultured↗