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PubMed1996

[Therapeutic experiences with extracorporeal photopheresis. Technical procedure, follow-up and clinical outcome in 31 skin diseases].

Extracorporeal photophoresis (ECP), a therapeutic modality that has been under investigation for some years, is based on separation of a leucocyte/lymphocyte-enriched cell fraction from the peripheral blood, extracorporeal treatment of the cells with 8-MOP/UVA and subsequent reinfusion of the cells in the patient. Its main effects seem to consist in changes to the immunologic behaviour of the photoinactivated/modulated cells. The immune response of the host is obviously stimulated by this treatment. ECP is normally performed for 4 h per day on 2 consecutive days every 4 weeks. The treatment is well tolerated and causes few side effects. In our department, 1210 ECP treatments were administered to 41 patients between 1990 and 1994 and a preliminary evaluation was performed. These patients included 21 with cutaneous T-cell lymphoma (CTCL), 10 with progressive systemic scleroderma, 4 with chronic graft-versus-host disease and 1 each with pemphigus vulgaris, epidermolysis bullosa acquisita, lupus erythematosus and cutaneous mucinosis. Patients with erythroderma and preserved immunocompetence achieved the best responses of all patients with CTCL. A treatment combining ECP with rIFN-alpha, PUVA and/or radiation was also successful in patients with tumour-stage CTCL and lymph node involvement. Progressive systemic scleroderma responded in more than 50% of our cases. Treatment results were impressive in 4 patients with chronic graft-versus-host disease presenting with sclerodermatous and lichenoid changes of the skin and mucous membranes. A clear improvement was also observed in the patient with pemphigus vulgaris refractory to standard therapies and in another patient with scleromyxoedema (Arndt-Gottron syndrome). The effectiveness of ECP seems to be quite well established in CTCL, but remains to be examined in autoimmune dermatoses. ECP is an attractive addition to the dermatological therapies available but our experience is still preliminary.

Adult↗
PubMed1996

Case report: insulin-dependent diabetes mellitus in childhood associated with scleroderma.

A 14-year-old boy presented with Type I diabetes mellitus and subsequently developed pancreatic exocrine insufficiency and systemic sclerosis (SSc). His diabetes had been diagnosed when he was about 5 years old, after the onset of ketoacidosis. Insulin treatment was provided from then until the time he was treated in our department. Exocrine pancreatic insufficiency, which occurred at age 9, was treated with pancreatic extracts. Cystic fibrosis was excluded after a chloride sweat test. The diagnosis of SSc was confirmed at age 14 on the basis of skin sclerosis, sclerodactyly and oesophageal and pulmonary involvement and then at age 18 by the occurrence of Raynaud's disease. Thus, this patient demonstrated a rare and previously unreported association of Type I diabetes mellitus and systemic scleroderma. Limited joint mobility and skin abnormalities are frequent in childhood diabetes mellitus but should not be misdiagnosed as systemic scleroderma.

Adolescent↗
PubMed1997

Evaluation of esophageal passage in selected connective tissue diseases.

The objective of this study was to determine whether, as in with other types of connective tissue diseases, there exists esophageal passage dysfunction as it exists in systemic scleroderma. It was also interesting to establish whether there is any correlation between this kind of dysfunction and the subjective complaints connected with the esophagus as well as the occurrence of Raynaud's phenomenon. Evaluation of the esophageal passage was performed on the basis of scintigraphic examination. The delay of esophageal passage occurs, besides systemic scleroderma, in other types of connective tissue diseases. This examination is a valuable test, thanks to which changes in the esophagus can be discovered, despite the lack of subjective complaints. In addition to systemic scleroderma, there is no close correlation between dysphagia and Raynaud's phenomenon.

Adult↗
PubMed1977

5-Hydroxytryptamine and tryptamine pathways in scleroderma.

Levels of 5-hydroxyindoleacetic acid, indoleacetic acid and total indoles were determined in the urine of 23 patients with systemic scleroderma and 7 patients with cutaneous scleroderma, before and after peroral loading with L-tryptophan (0-1 g/kg body weight). Before loading, 5-hydroxyindoleacetic acid levels were normal in nearly all cases of systemic scleroderma as well as of cutaneous scleroderma; however after loading, in nearly one half of cases there was no normal increase of this metabolite. These results suggest impaired transformation of serotonin into 5-hydroxyindoleacetic acid. A disproportionately high ratio of total indoles to indoleacetic acid suggests the presence of excess of tryptamine. The results of the study may indicate that in scleroderma metabolism of biogenic amines derived from tryptophan is abnormal, probably as a result of impaired activity of monoamine oxidase.

Adolescent↗
PubMed1975

Immunochemical characterization of the anti-RNA antibodies found in scleroderma and systemic lupus erythematosus. I. Differences in reactivity with Poly (U) and Poly-(A) Poly (U).

In a previous study, all 40 sera from patients with scleroderma, 20 of 40 sera from SLE patients, but none of 40 sera from normal controls, were found to have antibodies to ssRNA. All scleroderma sera were also found to react with HSA-coupled uridine and UMP and their reaction with HSA-coupled uridine and UMP and their reaction with ssRNA could be inhibited by uracil, uridine, and UMP. To characterize further these uracil-specific anti-RNA antibodies found in scleroderma and compare them with the anti-RNA antibodies found in SLE, we tested their reactivity with Poly (U) and with Poly (A)-Poly (U) and all but one failed to react with Poly (A)-Poly (U). This same serum was the only one in which the reaction with Poly (U) could not be inhibited with uracil. Reactivity of SLE sera was strikingly different from that found in scleroderma sera. Seventeen of 34 SLE sera studied reacted with ssRNA but only four of these reacted with Poly (U). Conversely, two SLE sera that reacted with Poly (U) did not react with ssRNA. Fifteen reacted with Poly (A)-Poly (U) and only two of these failed to react with ssRNA. Five SLE sera which were reactive with ssRNA did not precipitate with Poly (A)-Poly (U). All SLE sera which reacted with Poly (U) could be inhibited with uracil, although less effectively than in scleroderma. Reactivity with Poly (A)-Poly )U) was not inhibited with uracil nor with adenosine. These findings confirm that antibodies to RNA that are found in scleroderma are directed to uracil and thus specific to ssRNA, whereas RNA antibodies found in SLE sera are heterogeneous and directed to either the base, to the site of union of the base and sugar moiety to the ribose backbone, or to the helical structure of double stranded RNA. These differences and the respective antigenic specificities of these anti-RNA antibodies found in scleroderma and SLE may be theoretically important.

Adenine↗
PubMed1984

Fine structural capillary changes and basal lamina thickening in scleroderma (progressive systemic sclerosis) and Raynaud's disease.

Capillary abnormalities were found by electron microscopy in labial salivary gland biopsies obtained from 20 patients with progressive systemic sclerosis (PSS). They consisted of marked thickening and multilayering of basal lamina, degenerative and adaptative endothelial cell changes, alterations of pericytes and perivascular mononuclear cell infiltration. There was no correlationship between the intensity of the capillary abnormalities and the duration of the disease. In 12 patients with Raynaud's disease (RD) such changes were not found. Thus capillaries displayed normal-looking ultrastructural appearances. Mean capillary basal lamina width was consistently thicker in the PSS group than in the RD group (p less than 0.001). These observations are consistent with the hypothesis that the primary event in PSS occurs in the microvessels. It is suggested that labial salivary gland biopsy may be helpful for early recognition of PSS among subjects with Raynaud's syndrome.

Adolescent↗
PubMed1992

Association of amino acid sequences in the HLA-DQB1 first domain with antitopoisomerase I autoantibody response in scleroderma (progressive systemic sclerosis).

Previous studies in Caucasians with progressive systemic sclerosis (PSS) have suggested associations of antitopoisomerase I (antitopo I) autoantibodies with either serologically defined HLA-DR2 or DR5. To better define class II HLA associations with the antitopo I response, 161 PSS patients (132 Caucasians and 29 American blacks) were studied for antitopo I autoantibodies by immunodiffusion and immunoblotting, and their HLA-DRB1, DRB3, DQA1, and DQB1 alleles were determined by restriction fragment length polymorphic analysis and DNA oligotyping. Among Caucasians with antitopo I, HLA-DR5(DRB1*1101-*1104), DRB3*0202 and DQw3 (DQw7,8,9) were significantly increased in frequency. In American blacks, however, only HLA-DQB1*0301(DQw7) was significantly increased. The presence of HLA-DQB1*0301(DQw7) and other HLA-DQB1 alleles bearing the uncharged polar amino acid residue tyrosine at position 30 of the outermost domain was found in all antitopo I-positive Caucasian PSS patients compared with 66% of antitopo I-negative PSS patients (pc = 0.007) and 70% of normal controls (pc = 0.008), as well as all antitopo I-positive black patients. The association with HLA-DQB1 was independent of HLA-DR5(DRB1*1101-*1104) or any other HLA-DRB1, DRB3, or DQA1 alleles. Alternative or additional candidate epitopes for this autoimmune response include alanine at position 38 and threonine at position 77 of these same DQB1 alleles. These data suggest that genetic predisposition to the antitopo I response in PSS is associated most closely with the HLA-DQB1 locus.

Alleles↗
PubMed1984

[Prognosis and treatment of scleroderma].

The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.

Adrenal Cortex Hormones↗
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↗
PubMed1982

A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.

To define features of patients with Raynaud phenomenon that predict the evolution of connective tissue disorders, a prospective study was initiated. Patients with history or physical evidence of Raynaud phenomenon without causal or associated disorders underwent initial multisystem evaluation. Scleroderma (systemic sclerosis) of less than five years' duration was included for comparison. Patients were classified as having Raynaud phenomenon "only," undifferentiated connective tissue syndrome or scleroderma. Nailfold capillary microscopy was performed, and patterns were scored blindly from coded photographs. Of 91 patients with Raynaud phenomenon entered (Raynaud phenomenon only, n = 49; undifferentiated connective tissue syndrome, n = 22; scleroderma, n = 20), abnormal "scleroderma pattern" capillaries were noted in seven of 49, 19 of 22 and 19 of 20, respectively (p less than 0.005). Of 39 patients with Raynaud phenomenon only followed (mean duration, 23.7 months) undifferentiated connective tissue syndrome developed in three. Of 17 patients with undifferentiated connective tissue syndrome followed (mean duration, 27.6 months), six patients had transitions (four scleroderma; one scleroderma-systemic lupus erythematosus overlap; one SLE). Nailfold capillary abnormalities best identified transitional patients in both groups (eight of nine) and were more sensitive than ANA (six of nine), presence of digital ulcers (four of nine) or decreased esophageal motility (two of nine). This prospective study documents a useful role for capillary examinations in evaluating Raynaud phenomenon. Abnormal capillaries indicate an increased risk for connective tissue disease; normal capillaries favor idiopathic Raynaud phenomenon.

Capillaries↗
PubMed1978

Collagen in the cellular and fibrotic stages of scleroderma.

The collagen in localized and systemic scleroderma skin was studied by light microscopy with silver impregnation (50 patients), electron microscopy (14 patients), and immunofluorescence microscopy using specific antibodies against Type I and Type III collagens (12 patients). In the cellular stage, the dermis and adipose tissue revealed perivascular or diffuse cellular infiltrates (mostly lymphocytes, plasma cells, and macrophages), accompanied by deposition of Type III collagen. The lower dermis also showed an increase in Type III collagen. In the fibrotic stage, the papillary layer showed a reduction and/or clumping of Type III collagen as compared to normal skin. The lower dermis and the adipose tissue revealed compact collagen consisting exclusively of Type I collagen or a mixture of Type I and Type III collagen. The pattern of Type III collagen distribution was similar to that of reticulin, thus suggesting that at least some reticulin fibrils may represent Type III collagen.

Adipose Tissue↗
PubMedDate not supplied

Chromosomal alterations in scleroderma.

To bring some new arguments in support of the genetic theory regarding the etiology and pathogenesis of scleroderma 12 patients (2 males and 10 females, ranging in age between 13 and 64 years) with both localized and systemic scleroderma were studied. For each patient chromosomal preparations were made in cell cultures from peripheral blood. The structural cytogenetic aberrations, observed in proportion of 81.81%, were, in the order of frequency: chromatid breakage, gaps, acentric fragments, deletions. The alterations appeared both in the systemic scleroderma and in the localized one but cannot represent a factor of prognosis for the disease. The association of Raynaud's syndrome with cytogenetic aberrations may lead to an early diagnosis of the disease.

Adolescent↗
PubMed1979

The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma).

The CREST syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the CREST syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.

Adult↗
PubMed2002

Bullous lesions in scleroderma.

BACKGROUND: The occurrence of bullous lesions in localized or systemic scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases. OBJECTIVE: To investigate the frequency, clinical, and immunopathologic features of patients with scleroderma and bullous eruptions and to review the literature regarding this rare condition. METHODS: A retrospective study of 53 cases of scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases. RESULTS: Four of 53 patients exhibited bullous lesions in association with scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic scleroderma. CONCLUSIONS: Of the 53 original patients, we have described four cases of bullous scleroderma (7.5%) Illustrating several pathogenetic mechanisms of bulla formation. inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying scleroderma.

Adult↗