Foot Diseases ; etiology ; Humans ; Scleredema Adultorum ; Scleroderma, Systemic ; complications ; Skin

A 38-year-old woman with systemic lupus erythematosus had developed a cutaneous lesion of linear scleroderma on the forehead 4 years after the diagnosis of lupus erythematosus. This case of coexistence of the two diseases can be regarded as a clinical variant of the connective tissue disease-overlap syndrome.
Adult ; Female ; Humans ; Korea ; Lupus Erythematosus, Systemic/*complications ; Scleroderma, Localized/*complications

Systemic sclerosis (SS) is a connective tissue disease and cardiac involvement is common. Primary cardiac involvement such as conduction system disturbances and arrhythmias can also occur. However, reports of sustained ventricular tachycardia (VT) are rare. We report a case of catheter ablation of sustained ventricular tachycardia in a patient with systemic sclerosis using a conventional mapping system. A 64-yr-old woman with a 10-yr history of SS was referred for management of her ventricular tachycardia. There was no structural abnormality in cardiac chambers. However, electrophysiologic study revealed electrical substrate of ventricular tachycardia which could be ablated with pacemapping and substrate mapping. This case demonstrated successful conventional mapping and catheter ablation in a hemodynamically unstable patient with SS.
*Catheter Ablation ; Electrocardiography ; Female ; Humans ; Middle Aged ; Scleroderma, Systemic/*complications/*diagnosis ; Tachycardia, Ventricular/*etiology/physiopathology/*surgery

The majority of patients with scleroderma have gastrointestinal involvement, and a few experience gastrointestinal hemorrhage, however, gastrointestinal hemorrhage due to Mallory-Weiss syndrome is very rare. We report upon a 24-year-old pregnant woman with scleroderma who had gastrointestinal hemorrhage due to Mallory-Weiss syndrome.
Adult ; Female ; Gastrointestinal Hemorrhage/diagnosis/*etiology ; Human ; Mallory-Weiss Syndrome/*diagnosis/*etiology ; Pregnancy ; Pregnancy Complications/*diagnosis ; Scleroderma, Systemic/*complications

OBJECTIVE: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. METHODS: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters. RESULTS: Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array. CONCLUSION Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc.
Adult ; Aged ; Capillaries ; Female ; Hemorrhage ; Humans ; Lung Diseases, Interstitial/complications* ; Male ; Microscopic Angioscopy ; Middle Aged ; Nail Diseases/complications* ; Nails ; Raynaud Disease ; Scleroderma, Systemic/complications*

Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.
Scleroderma, Systemic/*complications/enzymology/*immunology ; RNA Polymerase III/*immunology ; RNA Polymerase I/*immunology ; Korea ; Kidney Failure, Acute/*complications/enzymology/*immunology ; Humans ; Female ; Critical Care ; Autoantibodies/*blood ; Aged

A 54-year-old Korean male with scleroderma-like manifestation of primary systemic amyloidosis presented with firm cutaneous induration of face and distal extremities, subcutaneous induration of the trunk and proximal extremities, limited range of motion in all joints, hoarseness, and dysphagia. Monthly high-dose intravenous immunoglobulin (hdIVIg) was given (three treatments, each time administering 0.4 g/kg per day for five days), and both signs and symptoms began to improve. However, the quantitative analyses of serum protein did not improve. Therapeutic plasma exchange (TPE) was performed monthly to clear the elevated serum immunoglobulin, and after several treatments, their levels normalized and symptoms were maintained in the improved state for more than two years. To summarize, hdIVIg and TPE combination therapy may be used as a safe first-line treatment for patients with primary systemic amyloidosis presenting with symptomatic monoclonal gammopathy.
Scleroderma, Systemic/diagnosis/etiology/*therapy ; *Plasma Exchange ; Middle Aged ; Male ; Immunoglobulins, Intravenous/administration & dosage/*therapeutic use ; Humans ; Combined Modality Therapy ; Amyloidosis/*complications

OBJECTIVETo assess the occurrence of stressful life events in the year before the initiation of systemic sclerosis.

METHODSA consecutive series of 40 patients with systemic sclerosis (mean age (56.3+/-11.9) years, mean disease duration (4.3+/-3.1) years; 32 females and 8 males), including 28 with diffuse cutaneous scleroderma and 12 with limited cutaneous scleroderma, were evaluated. A control group of 40 healthy subjects free of systemic sclerosis also was included. Socioeconomic status was investigated and Paykel's interview for recent life events (a semi-structured research interview covering 64 life events) was conducted.

RESULTSPatients with systemic sclerosis showed higher percentages of lower education (72.5%) and working class (82.5%), and reported more stressful life events (P<0.05), such as exits (P<0.05), undesirable events (P<0.01), and uncontrolled events (P<0.001), when compared with the control. More events that had an objective negative impact (P<0.001) were also reported in systemic sclerosis patients than in the control. These results are in accordance with a multifactorial model of pathogenesis in systemic sclerosis.

CONCLUSIONWe reported a strong relationship between stressful life events and the initiation of systemic sclerosis. Our findings are consistent with current understanding of the extensive links of behavioral responses to stress with neurophysiological and biochemical processes.

Female ; Humans ; Interviews as Topic ; Life Change Events ; Male ; Middle Aged ; Scleroderma, Systemic ; etiology ; pathology ; psychology ; Socioeconomic Factors ; Stress, Psychological ; complications ; pathology ; psychology

Scleroderma is a kind of connective tissue disease characterized by skin and other systems fibrosis. The underlying mechanisms for this disease are poorly understood due to its complexity. It is very difficult for scleroderma patient to heal the wound due to the special pathological characteristic of scleroderma.
Combined Modality Therapy ; methods ; Humans ; Negative-Pressure Wound Therapy ; methods ; Scleroderma, Systemic ; complications ; surgery ; Skin ; Skin Transplantation ; methods ; Warts ; therapy ; Wound Healing

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antinuclear/blood ; Esophageal and Gastric Varices ; Female ; Gastrointestinal Hemorrhage ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Raynaud Disease/diagnosis ; Scleroderma, Systemic/complications/*diagnosis ; Skin/pathology