Ocena morfologii i dystrybucji naczyń włosowatych w kapilaroskopii wału paznokciowego u chorych na twardzinę układową i zdrowych osób

Introduction: Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular damage and immunological abnormalities leading to fibrosis that can damage multiple organs. The pathogenesis is complex and still poorly understood. However impaired angiogenesis in SSc has a major role in tissue injury and sequelae fibrosis. Nailfold capillaroscopy (NC)/nailfold videocapillaroscopy (NVC) is safe and non-invasive methods used to investigate microvascular changes in the peripheral circulation and it is a method of great diagnostic value in diagnosing and monitoring the patients with SSc. Typical microvascular alterations, called scleroderma pattern characterized by giant capillaries, haemorrhages and successive loss of capillaries, are observed at NC/NVC in a significant percentage of SSc patients, hence our interest was focused on the assessment of NVC in patient with systemic sclerosis (SSc). Material and methods: Thirty patients with SSc according to the ACR and EULAR criteria and healthy volunteers underwent NVC assessment. Nailfold capillaroscopy was performed by a videocapillaroscope and the picture of the capillaries at the hands were documented and evaluate. Results: NVC disturbed patterns were significantly prominent in SSc patients (p < 0.05) compared to the healthy control group. A normal capillaroscopic pattern was not observed in patients with SSc. The number of loops/mm was significantly lower in SSc group (p < 0.05) and was 4.28 capillaries/mm (min.1/mm; max. 10/mm). We did not notice significant difference in frequency of mega-capillaries (lcSSc/dcSSc: 41%/29%, p > 0.05) and avascular areas (lcSSc/dcSSc: 64%/57%, p > 0.05) between limited (lSSc) and diffuse (dSSc) SSc. Conclusions: Severe capillary damage is characteristic for SSc patients therefore NVC seems to be useful for selection of patients developing SSc

Correlation of Endostatin and Tissue Inhibitor of Metalloproteinases 2 (TIMP2) Serum Levels With Cardiovascular Involvement in Systemic Sclerosis Patients

Fibrosis of oesophagus, lungs, heart, and kidney in the course of systemic sclerosis (SSc) may lead to dysfunction of the above organs or even patients death. Recent studies point out the role of angiogenesis and fibrosis disturbances in the pathogenesis of SSc. Heart fibrosis is one of the most important prognostic factors in SSc patients. So, the aim of our study was to examine cardiovascular dysfunction in SSc patients and its correlation with serum levels of vascular endothelial growth factor (VEGF), endostatin, and tissue inhibitor of metalloproteinase 2 (TIMP2). The study group comprised 34 patients (19 with limited scleroderma (lSSc) and 15 with diffuse scleroderma (dSSc)). The control group consisted of 20 healthy persons, age and sex matched. Internal organ involvement was assessed on the basis of specialist procedures. Serum VEGF, endostatin, and TIMP2 levels were evaluated by ELISA. We found cardiovascular changes in 15 patients with SSc (8 with lSSc and 7 with dSSc). The observed symptoms were of different characters and also coexisted with each other. Higher endostatin serum levels in all systemic sclerosis patients in comparison to the control group were demonstrated (P < .05). Also higher serum levels of endostatin and TIMP2 were observed in patients with cardiovascular changes in comparison to the patients without such changes (P < .05). The obtained results support the notion that angiogenesis and fibrosis disturbances may play an important role in SSc. Evaluation of endostatin and TIMP2 serum levels seems to be one of the noninvasive, helpful examinations of heart involvement in the course of systemic sclerosis

Chronic mucocutaneous candidiasis with endocrinopathy – case report

Chronic mucocutaneous candidiasis (CMC) is characterized by Candida infection of the mucous membrane, scalp, skin and nails. We present a case of a 42-year-old man who was treated twice in the Dermatological Department. He was admitted the first time as a 7-year-old boy because of skin and mucosal lesions and then the diagnosis of granuloma candidamyceticum was established. Thirty-one years later he was admitted again with a history of facial skin lesions and blepharitis. For a couple of years he had suffered from diabetes and hypothyroidism. The diagnosis of CMC with endocrinopathy was established in our patient

Circulating angiostatin serum level in patients with systemic sclerosis

Introduction : Systemic sclerosis (SSc) is achronic connective tissue disease characterized by microangiopathy with inadequate angiogenesis. Angiostatin (AS) is a potent antiangiogenic factor specifically inhibiting proliferation and inducing apoptosis of vascular endothelial cells. Aim : To evaluate the level of angiostatin in the serum of patients with SSc. Material and methods : Serum levels of AS were measured in 20 SSc patients and 12 healthy controls. Results : A statistically significant difference in the serum levels of AS in SSc patients was observed compared to the control group (636.51 vs. 869.20 ng/ml; p = 0.012). Significant correlations between limited and disseminated SSc (lSSc/dSSc) were not found, however, a difference between lSSc and the control group was demonstrated (620.00 vs. 869.20 ng/ml; p = 0.011). The serum level of AS was not associated positively with organ changes caused by SSc. However, a statistically significant lower serum level of AS was observed in patients with SSc and no esophageal (p = 0.008) or pulmonary changes (p = 0.007) compared to the control group. Conclusions : Our results reveal significant differences in AS level in SSc patients compared to the healthy controls, and suggest that a low level of AS may occur as a result of impaired angiogenesis

The use of isotretinoin in low doses and unconventional treatment regimens in different types of acne: a literature review

High effectiveness of isotretinoin treatment for severe types of acne resistant to antibiotics has been widely recognized. However, the recommended doses in conventional therapy, according to consensus of the Polish Dermatological Society, may cause serious adverse effects. Thus, research into less stressful, alternative treatment regimens with the use of low doses of isotretinoin has been carried out. The aim of the paper was to review the selected papers where authors present the results of their studies on different regimens with the use of isotretinoin in low doses in patients with acne, evaluate their efficacy, patient satisfaction, frequency of adverse effects, recurrences and also treatment costs

Acroosteolysis of distal phalanges in patient with systemic sclerosis – case report

Introduction. Acroosteolysis is a recognized, but under-researched and forgotten manifestation of systemic sclerosis (SSc). Objective . To present a case of exuberant acroosteolysis and subcutaneous tissue calcinosis in the course of SSc. Case report. The patient, aged fifty-three with limited systemic sclerosis (lSSc) for 8 years duration, was under dermatological treatment because of ulcerative inflammation of the 2nd and 4th finger of the right hand. Progressive acroosteolysis of the distal phalanx with calcinosis and severe digital ischaemia was diagnosed. Conclusion . There is growing evidence suggesting that acroosteolysis is an important manifestation of SSc due to the correlation with progressive microangiopathy and severity of digital ischaemia

The Role of Angiogenesis Factors in the Formation of Vascular Changes in Scleroderma by Assessment of the Concentrations of VEGF and sVEGFR2 in Blood Serum and Tear Fluid

Systemic sclerosis (SSc) is a connective tissue disorder characterized by tissue hypoxia, excessive fibrosis of skin and internal organs, and angiogenesis imbalance. The aim of the study was to evaluate in SSc patients the association between the retinal microcirculation disturbances and the presence of peripheral trophic changes and to determine the role of angiogenesis factors in the formation of vascular changes in scleroderma. Twenty-five SSc patients and 25 age- and sex-matched healthy controls were included to the study. Assay of vascular endothelial growth factor (VEGF) and soluble VEGF receptor-2 (sVEGFR-2) in blood serum and tears was done for all patients and controls using enzyme-linked immunosorbent assay. Retinal blood circulation was investigated with fluorescein angiography (FA) in the SSc patients only. In our research, proportion of mainly hypertensive patients presenting with a large spectrum of retinal microvascular lesions was 72%, while proportion of patients with skin microvascular lesions within distal phalanxes of fingers and toes was 76%. We noticed that patients with pathological changes in the FA examination had finger ulcerations significantly more often than patients without changes in the eye fundus. There were no statistically significant differences in the serum concentration of VEGF and sVEGFR2 between subjects in both analyzed groups. Analysis of lower levels of VEGF (p=<0.001) and sVEGFR-2 (p=<0.001) in blood serum accompanied by simultaneous higher levels of VEGF/sVEGFR-2 ratio in tears of SSc patients, as compared with the control group, indicates the superiority of proangiogenic factors in patients’ tears

Genotype and haplotype analysis of ABCB1 at 1236, 2677 and 3435 among systemic sclerosis patients

Systemic sclerosis (SSc) belongs to the group of systemic diseases of the connective tissue, which are characterized by a chronic autoimmune inflammatory process. P-glycoprotein, initially associated with the drug resistance in patients with cancer, becomes more and more often a subject of considerations in terms of its significance in the development of illnesses, including autoimmune diseases. The aim of the study was an attempt to answer the question whether there was a relationship between ABCB1 polymorphisms and morbidity of systemic sclerosis in a Polish population. The study was carried out in 61 patients with SSc and 100 healthy volunteers. Determination of polymorphisms C1236T and C3435T in ABCB1 was carried out with the PCR-RFLP (polymerase chain reaction – restriction fragment length polymorphism) method. The G2677T/A ABCB1 polymorphism was analysed with the allele-specific PCR method. No statistically significant differences were observed in the frequencies of ABCB1 genotypes and alleles between SSc patients and the control group. It was observed that haplotype 1236 C-2677 G-3435 T occurred in the group of patients with SSc statistically more frequently than in the group of healthy volunteers (25% vs. 15%; p = .032). Carriers of the haplotype demonstrated almost a twofold greater risk of SSc (OR = 1.85; p = .032). No statistically significant correlations for the other nine haplotypes were found. Presented results concerning the relationship of ABCB1 polymorphisms with susceptibility to systemic sclerosis are the first ones that were obtained in a Polish population. They imply that single nucleotide polymorphisms do not affect the risk for SSc, but the 1236 C-2677 G-3435 T haplotype might increase this risk