AB0769 Scleroderma mimics in cohort from an eustar centre (original) (raw)
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Cutaneous Manifestations of Scleroderma: A Case Report
Asian Journal of Health Research
Background: Scleroderma or systemic sclerosis is a rare multisystemic autoimmune disease characterized by vasculopathy, inflammation, and progressive fibrosis of the skin and multiple organs. Cutaneous manifestations and Raynaud’s phenomenon, usually becomes the initial presentation of scleroderma which noticed by the patient. Case: A 47-year-old-women presented with hardened and thickened skin on her arms and legs for 3 years which spreaded to her face and trunk. The lesions initially appeared as multiple red patches which progressed into white, thick and hard patches. The patient had history of recurrent Raynaud's phenomenon, dry cough, and shortness of breath. In the physical examination, the patient had skin hardening with salt and pepper appearance on her upper back and chest. The thoracic CT scan revealed interstitial lung disease. The patient diagnosed as definitive scleroderma based on The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) ...
Case Report LIMITED CUTANEOUS SCLERODERMA WITH AN UNUSUAL PRESENTATION-A CASE REPORT
2016
Systemic sclerosis or scleroderma (SSc) is a chronic multisystem disease of unknown etiology characterized by skin induration and thickening, accompanied by fibrosis and chronic inflammatory infiltration of internal organs, microvascular damage and dysfunction, and immune dysfunction. Systemic sclerosis is an uncommon acquired connective tissue disorder characterized by an abnormal thickening of the skin. Long history of Raynaud's phenomenon, Limited skin involvement (peripheral only), Calcification, telangiectasia, digital ischemia, pulp loss, pitted scars, paronychia, late onset of pulmonary hypertension, Capillary dilatation visible in nail folds and Anticentromere antibody positive are seen. We report this case of Limited Cutaneous Scleroderma with an unusual presentation.
Rheumatology, 2005
43 UI/ml (n<30). Anti-double-stranded DNA antibodies were negative. Histological examination of a skin biopsy demonstrated vacuolar degeneration of basal keratinocytes with sparse keratinocyte necrosis and a perivascular inflammatory infiltrate of lymphocytes in the superficial dermis. Direct immunofluorescence showed IgM and C3 deposits along the dermal-epidermal junction. Leflunomide was stopped without washout procedure. The patient was treated for 1 month with topical corticosteroids. Cutaneous eruption totally cleared within 3 weeks and anti-Ro antibodies reverted to normal (21 UI/ml in December 2004). Patient 2 was a 56-yr-old woman who had had erosive RA refractory to multiple agents for 9 yr. In April 1999, after a 100 mg loading dose for 3 days, a 20 mg daily leflunomide treatment was begun, with rapid clinical improvement. In February 2000, the patient presented a papulosquamous eruption localized on the ears, upper back and cheeks. No abnormal biological laboratory findings were observed: ANA, anti-double-stranded DNA antibodies and anti-histone antibodies were negative. Immunofluorescence analysis of a skin biopsy showed a lupus band along the dermal-epidermal junction. After leflunomide discontinuation and without a washout procedure, rapid and complete improvement of cutaneous features was observed. No recurrence of the SCLE was observed after a 3-yr follow-up period. Clinical, biological and immunohistological features of the two patients were suggestive of SCLE. These two patients with leflunomide-induced SCLE had no history of cutaneous eruption or clinical features of lupus erythematosus. These two cases of SCLE were most likely due to leflunomide since the eruption completely cleared after stopping leflunomide while other concomitant drugs were continued. In both cases, leflunomide was used as monotherapy without combination with MTX or biological agents. These two cases are reminiscent of a previous similar observation reported by Kerr et al. [5]. SCLE appeared 6 weeks after the introduction of leflunomide. Biological abnormalities were detectable as Ro (>100 /ml) and antihistone antibodies. Histological examination of a biopsy of the rash showed marked basal cell liquefaction and a moderate dermal perivascular chronic infiltrate. Direct immunofluorescence revealed IgG in the papillary dermis. Cutaneous features completely healed 5 months after stopping the drug. Clinicians should be aware of this rare side effect of leflunomide since it requires stoppage of the drug and may thus interfere with the treatment of RA. Leflunomide may now be listed among gold salts and sulphasalazine [6] as DMARDs that could potentially induce cutaneous lupus. X.L.L. has worked as a consultant for Aventis. The other authors have declared no conflicts of interest.
Immunologic and nonimmunologic sclerodermal skin conditions - review
Frontiers in Immunology, 2023
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Open Journal of Rheumatology and Autoimmune Diseases, 2019
Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients' files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases; a Raynaud's phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine.
Rheumatology, 2020
Objective SSc and localized sclerosis (LoS) are considered clinically distinct entities. We describe herein the coexistence of SSc and LoS by both a systematic literature review and an observational cohort study of unselected SSc patients. Methods Original studies documenting the coexistence of SSc and LoS were identified in three electronic databases by means of a systematic literature search according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Additionally, the coexistence of SSc and LoS was studied in a prospective cohort of SSc patients visiting the Ghent University Scleroderma Unit for their yearly follow-up visit between January 2018 and January 2019. Results Five studies were finally included for quality appraisal and data extraction. The coexistence of SSc and LoS ranged between 2.4 and 7.4%. RP, scleroderma pattern on nailfold videocapillaroscopy (NVC) and the presence of SSc-specific antibodies were commonly observed in c...
A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma
Arthritis & Rheumatism, 2001
Objective. Early diffuse scleroderma (systemic sclerosis; SSc) has no proven treatment. This study was undertaken to examine the efficacy of methotrexate (MTX) in improving the skin and other disease parameters in early diffuse SSc. Methods. Seventy-one patients with diffuse SSc of <3 years' duration were enrolled in a multicenter, randomized, placebo-controlled, double-blind trial. Thirty-five patients were treated with MTX and 36 with placebo. Treatment was administered for 12 months. The primary outcome measures were skin score (as determined with 2 different indices) and physician global assessment. Results. At baseline, there were no statistically significant differences in skin scores, carbon monoxide diffusing capacity (DLCO), physician global assessment, or other secondary outcome measurements between the 2 treatment groups. At study completion, results slightly favored the MTX group (mean ؎ SEM modified Rodnan skin score 21.4 ؎ 2.8 in the MTX group versus 26.3 ؎ 2.1 in the placebo group [P < 0.17]; UCLA skin score 8.8 ؎ 1.2 in the MTX group versus 11.0 ؎ 0.9 in the placebo group [P < 0.15]; DLCO in the MTX group 75.7 ؎ 4.6 versus 61.8 ؎ 3.4 in the placebo group [P < 0.2]). In addition, physician global assessment results favored MTX (P < 0.035), whereas patient global assessment did not differ significantly between groups. When between-group differences for changes in scores from baseline to 12 months were examined using intentto-treat methodology, MTX appeared to have a favorable effect on skin scores (modified Rodnan score ؊4.3 in the MTX group versus 1.8 in the placebo group [P < 0.009]; UCLA score ؊1.2 in the MTX group versus 1.2 in the placebo group [P < 0.02]), but differences in the degree of change in the DLCO and physician global assessment were not significant. For the UCLA skin score, these differences in results were not statistically significant after adjustment for baseline differences in sex distribution and steroid use. Dropout rates were similar in the 2 groups. Conclusion. Although results of this trial demonstrated a trend in favor of MTX versus placebo in the treatment of early diffuse SSc, the between-group differences were small and the power to rule out falsenegative results was only 50%. Our findings do not provide evidence that MTX is significantly effective in the treatment of early diffuse SSc. Scleroderma (systemic sclerosis; SSc) is a connective tissue disease that causes fibrosis of the skin and visceral organs such as the lungs, gastrointestinal tract, and heart (1). There are 2 major forms of SSc: diffuse and limited. Limited cutaneous SSc (lcSSc) has been