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PubMed1991

Diffuse systemic sclerosis and related diseases in Thailand.

Diffuse systemic sclerosis and the related syndromes were diagnosed in 115 patients during 1977-1987. Sixty-nine patients were available for clinical evaluation and analysis. These included 46 patients with diffuse systemic sclerosis, 2 with limited cutaneous systemic sclerosis, 2 with linear scleroderma, 18 with overlap syndrome (OS), and one with primary Raynaud's disease. The major clinical manifestations among the diffuse scleroderma patients were the involvement of the following systems; cutaneous (93.3%), musculoskeletal (69.6%), gastrointestinal (54.3%), and pulmonary (43.3%) while renal involvement (4.3%) was less common. Antinuclear antibodies were found in 89.7% with predominating speckle pattern (86.2%) and anti Scl 70 antibody (40.6%). In contrast to the Western series, HLA-DR2 was significantly increased in our diffuse systemic sclerosis patients (p less than 0.01). Among the 18 OS patients, the involvement of pulmonary system (44.4%) was the major cause of morbidity and renal involvement (55.6%) was found frequently although it was clinically mild. In conclusion, our diffuse systemic sclerosis and related diseases patients seem to have milder clinical manifestations than those of the Western series.

Adult↗
PubMed1995

A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy.

BACKGROUND: Scleroderma is a systemic rheumatic disorder seen in a wide range of clinical specialties. AIMS: To establish the prevalence and mortality rates of scleroderma in South Australia (SA), to determine the relative frequency and characteristics of the three principal subsets (diffuse, limited and overlap), and to examine the role of nailfold capillaroscopy in subset identification and implied prognosis. METHODS: Outpatient and discharge diagnostic indexes from five major teaching hospitals in SA were reviewed between February 1987 and November 1993. A total of 215 patients with scleroderma were identified. Case notes of 115 of these patients were reviewed in order to validate scleroderma diagnosis, and subset characteristics such as sex, mean age at diagnosis, extent of skin involvement, internal organ involvement and serology were analysed. Fifty-two of these patients were then examined prospectively to confirm positive discharge diagnosis, and nailfold capillaroscopy was performed on these patients. RESULTS: The point prevalence of scleroderma in SA for 1993 was estimated to be 208/10 This figure is a conservative estimate and is higher than most other reported series. The female to male ratio was 4:1. The majority of patients had limited disease with a ratio of 6:1:1.6 limited vs diffuse vs overlap. Systemic involvement excluding the oesophageal components limited disease was found predominantly in the diffuse group. Autoimmune serology was positive in 90% of patients, with Scl-70 being more common in diffuse scleroderma, anti-centromere antibody (ACA) in the limited form and anti-ribonucleoprotein (RNP) in the overlap form. Nailfold capillaroscopy was useful in predicting disease-subtype as capillary dilatation was observed predominantly in limited disease, capillary dropout in diffuse disease. CONCLUSIONS: Scleroderma is more common in SA than previously recognised. Limited disease is more common than diffuse or overlap disease, carries a better prognosis and in associated with ACA. Nailfold capillaroscopy is a useful tool in disease assessment and may provide useful diagnostic and prognostic information.

Adolescent↗
PubMed2002

Coexistence of antitopoisomerase I and anticentromere antibodies in patients with systemic sclerosis.

BACKGROUND: Antibodies targeting DNA topoisomerase I (ATA) or centromere proteins (ACA) are associated with clinical subsets of patients with systemic sclerosis (SSc). The occurrence of those autoantibodies is considered to be mutually exclusive. OBJECTIVE: To describe the clinical and immunogenetic data of three patients who are co-expressing both antibodies, and then review previous publications. METHODS: Both antibodies were detected by different methods, including indirect immunofluorescence technique, enzyme linked immunosorbent assay, immunodiffusion, and immunoblot. Patients were HLA typed by serological and molecular genetic methods. Data were extracted from published reports for comparison. The search for published studies was through Medline and other database research programmes. RESULTS: During routine laboratory diagnostics over several years three patients with scleroderma and coincidence of ATA and ACA were identified: patient 1 with diffuse SSc, Raynaud's phenomenon, puffy fingers and fingertip necrosis, contractures, and calcinosis; patient 2 with diffuse SSc, Raynaud's phenomenon, oedema of the hands, and interstitial calcinosis of hands, knees, and shoulders, and pulmonary fibrosis; patient 3 with scleroderma of hands, forearms, and face, Raynaud's phenomenon, puffy fingers, finger contractures, fingertip necrosis, and calcinosis. All three patients studied were carriers of HLA alleles known to be associated with these autoantibodies. In serial measurements the concentrations of the two antibodies showed independent or even reverse fluctuations. Screening of 100 patients with ACA for ATA and vice versa disclosed no further patients with coincidence of these antibodies. Twenty eight cases of ACA/ATA coexistence in 5423 patients (0.52%) with SSc or SSc associated symptoms were found in an analysis of published studies. CONCLUSION: The expression of ATA and ACA is not totally mutually exclusive, but coincidence is rare (<1% of patients with SSc). Patients with both autoantibodies often have diffuse scleroderma and show immunogenetic features of both antibody defined subsets of SSc.

Adult↗
PubMedDate not supplied

[Differential diagnosis of diffuse eosinophilic fasciitis].

The authors studied clinical course, morphological findings, condition of microcirculation in 22 patients with diffuse fasciitis++ in comparison with 20 patients with systemic scleroderma and 20 patients with other similar diseases. Comparing clinico-laboratory data of 62 patients sharp differences were observed between diffuse fasciitis++ and systemic scleroderma. In diffuse fasciitis++ microcirculation disorders were less marked than in systemic scleroderma. According to the above research diffuse eosinophilic fasciitis++ is considered to be an independent nosologic form.

Diagnosis, Differential↗
PubMed1997

Symptomatic spinal calcinosis in systemic sclerosis (scleroderma).

Two patients with diffuse cutaneous systemic sclerosis and spinal calcification, involving the lumbar spine in one and the cervical spine in the other, are described. Computed tomography-guided aspiration of the calcific masses was performed, and material aspirated from one patient was shown to be apatite, Ca5(PO4)3OH. One patient showed improvement following lumbar laminotomy, hemilaminectomy, and diskectomy.

Calcinosis↗
PubMedDate not supplied

Lack of an association between diffuse systemic sclerosis and HLA-DR1 or HLA-DR5.

The HLA-DR locus of the major histocompatibility complex encodes class II molecules which participate in immune responses through regulation of T cell interaction with antigen presenting cells. Previous association studies between HLA-DR antigens and the autoimmune disease, systemic sclerosis (or scleroderma), have yielded conflicting results. Some investigators have reported an association between this disease and HLA-DR1, while others have demonstrated an association with HLA-DR5. In this study, we used restriction fragment length polymorphisms in the HLA-DR locus to compare allelic frequencies of HLA-DR1 and HLA-DR5 in scleroderma patients with diffuse disease and healthy control subjects. No significant difference in the allelic frequency of either antigen was observed between the groups. These results suggest that HLA-DR1 and HLA-DR5 antigens are unlikely to contribute significantly to disease susceptibility in scleroderma.

Alleles↗
PubMed2000

Increased phosphorylation of transcription factor Sp1 in scleroderma fibroblasts: association with increased expression of the type I collagen gene.

OBJECTIVE: To determine the potential roles of transcription factors Sp1 and Sp3 in the increased expression of the human alpha2(I) collagen gene in scleroderma fibroblasts. METHODS: Dermal fibroblasts from 7 patients with diffuse systemic sclerosis (SSc; scleroderma) of recent onset and from 7 healthy individuals were studied. The levels of expression of alpha2(I) procollagen, Sp1, and Sp3 messenger RNA (mRNA), with or without stimulation by transforming growth factor beta (TGFbeta) or oncostatin M (OSM), were evaluated by Northern blot analysis, and the respective protein levels were determined by immunoblotting. The DNA binding activity of nuclear proteins recognizing the cis-acting elements in the human alpha2(I) collagen promoter was examined by gel mobility shift assays. The levels of Sp1 phosphorylation were investigated by immunoprecipitation using an antiphosphoserine-specific antibody. RESULTS: SSc fibroblasts showed basal alpha2(I) collagen mRNA levels that were approximately 3 times higher than those in normal fibroblasts. TGFbeta or OSM increased human alpha2(I) collagen mRNA expression in normal dermal fibroblasts, but these cytokines failed to increase alpha2(I) collagen mRNA levels in SSc fibroblasts. There were no significant differences in the levels of expression of Sp1 or Sp3 between SSc and normal fibroblasts. However, increased Sp1 phosphorylation was detected in SSc fibroblasts compared with normal fibroblasts. Mithramycin, a specific inhibitor of Sp1 binding, abolished the increased expression of the alpha2(I) collagen gene in SSc fibroblasts, in a dose-dependent manner. CONCLUSION: These results demonstrate the involvement of Sp1 in the up-regulation of expression of the alpha2(I) collagen gene in SSc fibroblasts.

Collagen↗
PubMed1990

L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis.

OBJECTIVE: To assess the use of L-tryptophan by patients with eosinophilic fasciitis and compare this with its use by patients with progressive systemic sclerosis (scleroderma). DESIGN: Retrospective and prospective analysis. Six patients with eosinophilic fasciitis were identified retrospectively and two prospectively. Retrospective identification of patients was done by questioning hospital-affiliated rheumatologists and dermatologists and by searching the hospital dermatopathology database. The patients with scleroderma or morphea were prospectively identified by questioning consecutive office patients with these established diagnoses. SETTING: University of Pennsylvania rheumatology and dermatology practices. PATIENTS: Eight patients with eosinophilic fasciitis; 40 consecutive patients with scleroderma (27 with diffuse cutaneous and 13, limited cutaneous disease); 3 patients with morphea. RESULTS OF DATA ANALYSIS: All eight patients with eosinophilic fasciitis had taken L-tryptophan before the onset of their disease. All had myalgias and high peripheral eosinophil counts (most greater than 5000 cells/mm3). Only 1 of 40 patients with scleroderma (no patients with morphea) had used L-tryptophan preceding illness (P less than 0.001 compared with eosinophilic fasciitis). Six patients with eosinophilic fasciitis had taken L-tryptophan for less than 8 months. One patient had taken it for 9 years before developing skin induration. Two patients were newly identified as having hypothyroidism; two developed neuropathy; and two had severe flexion contractures (several occurring in areas without skin induration). Five patients had low-titer antinuclear antibodies, indicating a possible autoimmune process. Most patients had only a partial response to systemic corticosteroid therapy. One patient has had important disease regression in response to isotretenoin therapy that was evident even while she continued to take L-tryptophan. CONCLUSIONS: L-Tryptophan use can lead to eosinophilic fasciitis whereas it does not appear to cause classic scleroderma. The disease process does not automatically remit after discontinuation of L-tryptophan-containing products.

Adult↗
PubMed1984

[Lymphocytic interstitial pneumonia in Gougerot-Sjögren syndrome with scleroderma].

The authors report the case of a 70 year old woman with a 15 year history of the Gougerot-Sjogren syndrome, Raynaud's phenomenon and sclerodactyly. Progressive respiratory symptoms in this case were not due to pulmonary fibrosis secondary to scleroderma but to diffuse interstitial lymphocytic pneumonia (ILP) secondary to the Gougerot-Sjogren syndrome. The authors emphasize the diagnostic value of the significant, permanent lymphocytosis in the aspirate after broncho-alveolar lavage. The differential diagnosis is difficult because the radiological and clinical signs of pulmonary complications of the Gougerot-Sjogren syndrome and of scleroderma are very similar. Reports of the triple association of ILP-Gougerot-Sjogren syndrome-scleroderma are uncommon in the medical literature. However, prospective studies suggest that paraclinical stigmata of the Sjogren syndrome are relatively common in patients with scleroderma. These observations suggest that some patients with clinical "pulmonary fibrosis" may in fact have ILP secondary to slowly progressive Sjogren's syndrome.

Aged↗
PubMed2005

Classification of systemic sclerosis. Visions and reality.

Systemic sclerosis, scleroderma (SSc) is a disabling condition that shortens life expectancy. Disease heterogeneity and difficulties separating SSc from SSc-like conditions make classification an important issue. Limited cutaneous and diffuse cutaneous SSc, with different severity and survival, have been recognized for several years as distinct subsets. Some authors have suggested an intermediate cutaneous form with intermediate survival. This issue remains unsettled, however. The technique of capillaroscopy is helpful as an adjunct diagnostic tool to separate idiopathic Raynaud's phenomenon from SSc. Digitized video-capillaroscopy is developing as a powerful new method to assess individual capillaries over time. Using the simpler techniques of video-capillaroscopy, different patterns have been described and named 'early', 'active', 'late' and 'slow'. The value of nailfold video-capillaroscopy to distinguish different subsets or provide prognostic information for use in daily practice remains to be assessed. The features of CREST (calcinosis, Raynaud's, oesophagus dysmotility, sclerodactyly, telangiectasias) are not confined to single subsets of SSc. There is no convincing evidence of any advantage for distinguishing the limited, intermediate and diffuse forms of SSc rather than only the limited and diffuse forms.

CREST Syndrome↗
PubMed1998

Oral iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis: a multicenter, placebo-controlled, double-blind study.

OBJECTIVE: To evaluate the efficacy and tolerability of an oral preparation of iloprost, a prostacyclin analog, in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (scleroderma). METHODS: A multicenter, randomized, parallel-group, placebo-controlled double-blind study was performed at university and community-based medical centers. Patients were randomly assigned to receive either 50 microg of iloprost orally twice daily or an identical gelatin-coated capsule containing placebo for 6 weeks. Outcome measures included average total daily duration of RP attacks, average number of RP attacks, and RP condition scored via a standardized daily diary. RESULTS: Three hundred eight patients with scleroderma (272 women, 36 men, mean age 49 years [range 18-80]) were enrolled. One hundred fifty seven were assigned to receive iloprost and 151 to receive placebo. One hundred forty-three patients in the iloprost group (91.1%) and 144 in the placebo group (95.4%) completed the 6-week treatment phase. Fifteen of these treated patients (8 iloprost, 7 placebo) failed to complete all of the followup visits. The mean reduction in the average duration of attacks from baseline to week 5-6 was 24.32 minutes in the iloprost group and 34.34 minutes in the placebo group (P = 0.569). Likewise, the mean reduction from baseline to week 5-6 in the daily frequency of attacks was 1.02 in the iloprost group and 0.83 in the placebo group (P = 0.459). The Raynaud's condition score, a patient-completed assessment of the severity of RP attacks, was reduced by 1.32 in the iloprost group and 1.00 in the placebo group (P = 0.323). The lack of significant difference between treatment groups did not change when a variety of factors, including use of other vasodilators, duration of disease, classification of scleroderma (limited versus diffuse), or number of baseline digital ulcers were taken into account. Premature withdrawal from the study due to adverse events occurred in 10 patients (6.4%) in the iloprost group and 3 (2.0%) in the placebo group (P = 0.058). CONCLUSION: Oral iloprost at a dosage of 50 microg twice daily is no better than placebo for management of RP secondary to scleroderma, either during 6 weeks of treatment or during 6 weeks of posttreatment followup.

Administration, Oral↗
PubMedDate not supplied

Cytokines and fibrosis.

Inflammation is frequently associated with changes in the surrounding connective tissue. Inflammatory mononuclear cells (MNC) produce biologically active molecules, cytokines (CK), which may regulate the growth and function of connective tissue fibroblasts. In this article, we review the characteristics of lymphocyte and monocyte CK which appear to be involved in regulating fibroblast recruitment, proliferation, and matrix synthesis. Whereas these MNC products are important in normal physiologic wound healing, an imbalance of these CK may lead to pathophysiologic fibrosis and/or destruction of connective tissue components. Clinical states associated with MNC-mediated connective tissue pathology include scleroderma, rheumatoid arthritis, diffuse pulmonary idiopathic fibrosis, sarcoidosis and atherosclerosis. Characterization of the molecular pathways linking inflammatory mononuclear cells and fibrosis may provide avenues for therapeutic intervention.

Arthritis, Rheumatoid↗
PubMedDate not supplied

Autoantibodies in scleroderma.

In scleroderma a profusion of circulating autoantibodies have now been defined. They include autoantibodies to Scl-70 or DNA topoisomerase 1, and to centromere/kinetochore proteins of 17.80 and 140 kilodaltons. In addition, there are several antigens which are resident primarily in the nucleolus and they are RNA polymerase 1, PM-Scl, fibrillarin and 7-2 ribonucleoprotein. Antibody to Scl-70 has been found primarily in the diffuse form of scleroderma and antibody to the centromere/kinetochore proteins in the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia) subset of scleroderma. Autoantibodies to the nucleolar antigens RNA polymerase 1, PM-Scl, fibrillarin and 7-2 RNP have been detected in at least 10% of all patients with scleroderma. For several reasons which are discussed, it appears that the autoantibody response in scleroderma is antigen-driven and further that the autoantigens involved in this disease are present at some time in the nucleolus. These observations may be providing clues to some of the basic mechanisms initiating autoimmunity.

Autoantibodies↗
PubMed1999

Motor activity of the gallbladder in systemic sclerosis.

OBJECTIVE: We sought to measure gallbladder emptying in scleroderma patients, when stimulated by exogenous cholecystokinin. METHODS: Twenty-eight consecutive scleroderma patients were evaluated. Ten were excluded for the presence of gallstones. Gallbladder motor function was studied in 18 patients and 18 controls, using specific parameters for the quantification of gallbladder emptying dynamics. Resting gallbladder volumes were compared using the Dodds method with real-time ultrasound. Cholecystokinin (CCK)-stimulated gallbladder function (0.02 microg/kg CCK intravenous infusion/30 min) was assessed by a scintigraphic technique using 99mTc-DISIDA. Five patients presented with CREST syndrome, 13 with the diffuse form of scleroderma. Four were men, 14 women (average age = 46.6+/-15.4 yr). Patients and controls were paired by gender, age, and weight. RESULTS: Resting gallbladder volumes were larger in the four men with scleroderma than in the women with this disease (p < 0.03, Mann-Whitney). The mean gallbladder resting volume in scleroderma patients was not different from the mean volume detected among controls (p = 0.25), even when controlling for gender (p = 0.78 for women, p = 0.08 for men), scleroderma disease subtype (p = 0.50), or disease duration (p = 0.48). Latency period, ejection period, ejection rate, or ejection fraction as measured during cholecystokinin-stimulated scintigraphic studies were not significantly different between patients and controls. A trend was detected for reduction of the ejection period in scleroderma women (p = 0.70) when compared with scleroderma men. More than 35% of the scleroderma patients presented biliary lithiasis. CONCLUSIONS: There was no significant difference in gallbladder dynamics measured by a scintigraphic technique in scleroderma patients, compared with controls, when gallbladder motor function was evaluated by intravenous CCK.

Adult↗
PubMed2000

Prazosin for Raynaud's phenomenon in progressive systemic sclerosis.

OBJECTIVES: To determine the effects and toxicity of prazosin versus placebo proposed for the treatment of Raynaud's phenomenon (RP) in scleroderma. SEARCH STRATEGY: We searched the Cochrane Controlled Trials Register, and Medline up to December 1996 using the Cochrane Collaboration search strategy developed by Dickersin et al.(1994). Key words included: Raynaud's or vasospasm, scleroderma or progressive systemic sclerosis or connective tissue disease or autoimmune disease. Current Contents were searched up to and including April 7, 1997. All bibliographies of articles retrieved were searched and key experts in the area were contacted for additional and unpublished data. The initial search strategy included all languages. SELECTION CRITERIA: Randomized controlled trials comparing prazosin versus placebo were eligible if they reported clinical outcomes from the start of therapy. Trials with a greater than 35% dropout were excluded. Trials were included if patients with diffuse or limited scleroderma were the subjects. If patients with other connective tissue diseases or primary Raynaud's were included, the trial was used if the data on the scleroderma patients could be extracted from the paper. DATA COLLECTION AND ANALYSIS: All data were abstracted by two independent and trained reviewers (DF, AT), and verified by a third reviewer (JP). Each trial was assessed independently by the same two reviewers for its quality using a validated quality assessment tool (Jadad 1996). Peto's odds ratios were calculated for all dichotomous outcomes and a weighted mean difference was carried out on all continuous outcomes. Fixed effects and random effects model were used if the data was homogeneous or heterogeneous, respectively. MAIN RESULTS: Two trials with a total of 40 patients were included. Prazosin has been found in two randomized controlled cross-over trials to be more effective than placebo in the treatment of Raynaud's secondary to scleroderma. However, the positive response is modest and side effects are not rare in those taking prazosin. REVIEWER'S CONCLUSIONS: Prazosin is modestly effective in the treatment of Raynaud's phenomenon secondary to scleroderma.

Adrenergic alpha-Antagonists↗
PubMed2002

Association of 5'-untranslated region of the Fibrillin-1 gene with Japanese scleroderma.

Excessive production of extracellular matrix (ECM) constituents is a hallmark scleroderma or systemic sclerosis (SSc). Fibrillin-1, a major component of microfibrils in the ECM, may play a role in the pathogenesis of SSc. The TSK1 mouse model of SSc bears an in-frame duplication of the Fibrillin-1 gene (FBN1) which results in a larger than normal protein that is more susceptible to proteolysis. Metabolic labeling studies of Fibrillin-1 in human SSc dermal fibroblasts demonstrated that while normal amounts of Fibrillin-1 are synthesized, the protein itself appears to be unstable. Moreover, autoantibodies specific for Fibrillin-1 have been demonstrated in serum from SSc patients and TSK1 mice. In particular, a high frequency of anti-Fibrillin-1 was observed in Japanese patients with diffuse and limited scleroderma or CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome. Genetic studies in a Native American population with high prevalence of using microsatellite marker showed strong association between FBN1 haplotypes and SSc. Subsequently, studies of FBN1 single nucleotide polymorphisms (SNPs) demonstrated that certain FBN1 haplotypes were associated with SSc in both Native American and Japanese patients with limited scleroderma. Thus, FBN1 was sequenced in 22 Japanese SSc patients to ascertain the presence of any relevant mutations or SNPs. Sequence analysis revealed eight coding and 14 non-coding SNPs and other polymorphisms. Among them, a CT insertion in the 5'-untranslated region of exon A had a significant negative association with disease.

5' Flanking Region↗
PubMed2002

Antibody-mediated gastrointestinal dysmotility in scleroderma.

BACKGROUND & AIMS: Defects in enteric excitatory neurotransmission have been proposed to underlie the gastrointestinal dysmotility associated with scleroderma (systemic sclerosis). This study investigated whether patients with scleroderma produce antibodies that inhibit M3-muscarinic or neurokinin receptor-mediated intestinal contractions, either directly or via an effect on L-type voltage-gated calcium channels (VGCCs). METHODS: Responses of mouse colon longitudinal muscle to stimulation by the muscarinic agonist carbachol (1-300 micromol/L) and neurokinin-1 and -2 receptor agonists were measured in the absence and presence of serum (2%) or immunoglobulin G (IgG) (0.3-1.0 mg/mL) from patients with scleroderma, those with other autoimmune disorders, and healthy controls. The role of L-type VGCCs in carbachol- and tachykinin-evoked contractions was assessed using nicardipine. RESULTS: M3-muscarinic receptor-mediated contractions were inhibited by Ig fractions from 7 of 9 patients with scleroderma (limited and diffuse forms), 4 of 4 patients with primary Sjögren's syndrome, and 3 of 3 patients with secondary Sjögren's syndrome. Ig fractions from healthy controls did not inhibit the M3-muscarinic receptor-mediated contractions. Inhibition by Ig was concentration-dependent; a maximum inhibition of approximately 40% occurred at 0.6 mg/mL IgG. Both M3-muscarinic and neurokinin receptor-mediated contractions were L-type VGCC dependent. Patient sera had no effect on responses to neurokinin receptor stimulation, demonstrating the lack of antibodies inhibiting L-type VGCCs. CONCLUSIONS: Functional antibodies specifically inhibiting M3-muscarinic receptor-mediated enteric cholinergic neurotransmission may provide a pathogenic mechanism for the gastrointestinal dysfunction seen in patients with scleroderma.

Aged↗
PubMed1992

Papular mucinosis (scleromyxoedema) complicating diffuse systemic sclerosis: clinical features and electron microscope observations.

Papular mucinosis (scleromyxoedema) is an uncommon disorder characterized by generalized papular eruption and cutaneous induration, which may be associated with a variety of extracutaneous manifestations. Although scleroderma and papular mucinosis share many features, they are clinically and histologically distinct entities. We report here a patient with diffuse scleroderma who developed superimposed papular mucinosis. Degranulating mast cells were a prominent ultrastructural finding in the involved skin. The occurrence of scleroderma and papular mucinosis, two uncommon cutaneous indurative diseases, in the same patient has not been described previously.

Biopsy↗