Chlormethine gel is effective for the treatment of skin lesions in patients with early- and late-stage mycosis fungoides in clinical practice.

BACKGROUND: Chlormethine gel is a skin-directed therapy used for patients with mycosis fungoides (MF) that showed a favourable risk/benefit profile in a randomized clinical trial. Currently, data on chlormethine gel use in real-world settings are limited. OBJECTIVES: The aim of this study was to assess safety and efficacy of chlormethine gel treatment in patients treated during daily clinical practice and investigate associations between response and disease stage, lesion type, mono- or combination therapy, and occurrence of dermatitis. METHODS: Clinical data from patients using chlormethine gel from three sites in Greece were analysed. Efficacy was assessed through modified Severity-Weighted Assessment Tool (mSWAT) scores. Safety assessments included analysis of the occurrence and severity of dermatitis. The Skindex-29 questionnaire was used for quality-of-life assessments. RESULTS: Fifty-eight patients were included. The overall response rate (ORR) increased from 37.9% at month 1 to 80.8% at month 9. For 64.2% of patients, response was maintained for at least 4 months (ORR4). At month 3, a higher ORR was seen for patients with patches (69.7%) than patients with plaques/tumours (both 15.2%). A higher ORR4 was observed for patients with early- vs late-stage disease (71.4% vs. 36.4%) and patients on mono- vs combination therapy (75% vs. 47.6%). Dermatitis was observed in the majority of patients (72.4%), but the presence or severity of dermatitis was not directly correlated with treatment response. Both mSWAT and Skindex-29 scores decreased significantly during treatment, and changes in these scores from baseline to month 6 showed a positive correlation (r = 0.55, P = 0.026). CONCLUSIONS: Chlormethine gel was effective for the treatment of skin lesions in patients with early- and late-stage MF in clinical practice. Response rates increased over time, indicating that continued treatment with the gel is important. Dermatitis may be managed by reducing the treatment frequency; the occurrence of dermatitis did not affect the response to treatment.

Multicentric EORTC retrospective study shows efficacy of brentuximab vedotin in patients who have mycosis fungoides and Sézary syndrome with variable CD30 positivity.

BACKGROUND: Brentuximab vedotin (BV) was approved as a therapy for mycosis fungoides (MF) based on the ALCANZA trial. Little real-world data, however, are available. OBJECTIVES: To evaluate the efficacy and safety of BV in patients with MF/Sézary Syndrome (SS) with variable CD30 positivity in a real-world cohort and to explore potential predictors of response. METHODS: Data from 72 patients with MF/SS across nine EORTC (European Organization for Research and Treatment of Cancer) centres were included. The primary endpoint was to evaluate the proportion of patients with: overall response (ORR), ORR lasting over 4 months (ORR4), time to response (TTR), response duration (RD), progression-free survival (PFS) and time to next treatment (TTNT). Secondary aims included a safety evaluation and the association of clinicopathological features with ORR, RD and TTNT. RESULTS: All 72 patients had received at least one systemic treatment. ORR was achieved in 45 of 67; ORR4 in 28 of 67 with a median TTR of 8 weeks [interquartile range (IQR) 5·5-14] and with a median RD of 9 months (IQR 3·4-14). Median PFS was 7 months (IQR 2-12) and median TTNT was 30 days (6-157·5). Patient response, RD, PFS and TTNT were not associated with any clinicopathological characteristics. In the MF group, patients with stage IIB/III vs. IV achieved longer PFS and had a higher percentage of ORR4. There was a statistically significant association between large-cell transformation and skin ORR (P = 0·03). ORR4 was more frequently achieved in patients without lymph node involvement (P = 0·04). CONCLUSIONS: BV is an effective option for patients with MF/SS, including those with variable CD30 positivity, large-cell transformation, SS, longer disease duration and who have been treated previously with several therapies.

Effect of heterozygous beta thalassemia on HbA1c levels in individuals without diabetes mellitus: A cross sectional study.

AIMS: To investigate the effect of heterozygous ß-thalassemia on HbA1c levels in a population without diabetes mellitus (DM). METHODS: Using a cross-sectional design, HbA1c levels were compared between two groups of 100 consecutive carriers of ß-thalassemia and 100 healthy controls matched for age, gender and BMI, taking into account fasting serum glucose and fructosamine levels. The effect of hemoglobin concentration on HbA1c was also examined. RESULTS: The mean HbA1c level was almost identical in the two groups (33.6 mmol/mol [5.23%] vs. 33.6 mmol/mol [5.22%], p = 0.857). Within the group of ß-thalassemia, there was a positive correlation between HbA1c and hemoglobin concentration (r = 0.455, p < 0.001), which was not observed in controls. ß-thalassemia carriers without anemia had slightly higher HbA1c levels compared to those with anemia (34.9 mmol/mol [5.35%] vs. 32.5 mmol/mol [5.12%] p < 0.001, absolute difference (2.4 mmol/mol [0.23%]). In multivariable analysis, hemoglobin concentration, BMI and 1st degree family history of T2DM were significant predictors of HbA1c, while ß-thalassemia carrier state was non-significant (p = 0.07). CONCLUSIONS: In individuals without DM, heterozygous ß-thalassemia has a borderline effect on HbA1c levels, while the impact of ß-thalassemia trait-associated anemia on HbA1c is of negligible clinical significance. These findings advocate for the clinical use of HbA1c as a diagnostic criterion for diabetes mellitus in this population.

Prevalence of psoriatic arthritis and its correlates among patients with psoriasis in Greece: results from a large retrospective study.

OBJECTIVES: To evaluate the prevalence and its clinical characteristics of psoriatic arthritis (PsA) in a specialized psoriasis clinic of a University Hospital. METHODS: In this retrospective study, 278 patients with psoriasis were evaluated between 2011 and 2013. RESULTS: The study included 278 patients with psoriasis: 144 (52%) were male and 134 (48%) female. Their median age was 51.41 with median psoriasis presenting age of 34.52 years. Referring to the type of psoriasis, 86% presented with plaque psoriasis, 5% guttate, 2% palms and soles, 2% inverse, 1% pustular and 4% with psoriasis of more than one type. Nail disease appeared in 121 patients (43.5%) and scalp disease in 175 (63%). Of these patients, 85 (30%) had PsA, whereas 51% of patients with PsA had psoriatic nail disease. With reference to the PsA type, 43 (51%) patients presented with polyarthritis, 10 (12%) with oligoarthritis, 7 (8%) with axial arthritis, whereas the rest 25 of them (31%) had PsA of more than one type. The subgroup of patients with PsA had significantly higher rates of comorbidities including arterial hypertension, diabetes and hypercholesterolaemia compared to non-PsA patients with 41% vs. 17% (P = 0.001), 20% vs. 8% (P = 0.021) and 41% vs. 19% (P = 0.004), respectively. CONCLUSION: The prevalence of PsA among patients with psoriasis was relatively higher in Greece compared to other ethnic-based studies. Comorbidities related to life expectancy were more frequent. As there is a high percentage of undiagnosed cases with active arthritis among patients with psoriasis, dermatologists should be aware of PsA clinical signs in order to recognize it earlier and provide successful treatment.

Ustekinumab induces fast response and maintenance of very severe refractory scalp psoriasis: results in two Greek patients from the psoriasis hospital-based clinic.

BACKGROUND: Scalp psoriasis, one of the most common sites of psoriasis involvement, is often difficult to control with topical agents. There is a lack of substantial evidence-based data for the efficacy and safety of systemic therapies. METHODS: Two patients from our university-based psoriasis clinic with chronic plaque psoriasis and severe recalcitrant scalp involvement were assessed by Psoriasis Area and Severity Index and Psoriasis Scalp Severity Index scores, respectively, and quality of life by the Dermatology Life Quality Index. RESULTS: We report 2 psoriasis patients with very severe scalp psoriasis who developed a fast clinical response of scalp psoriasis to ustekinumab in 8 weeks with excellent patient adherence up to 28 weeks of follow-up and positive impact on quality of life due to rapid and long-term clearing. CONCLUSION: Ustekinumab produces a fast clinical response of recalcitrant scalp psoriasis with excellent patient adherence and a positive impact on quality of life due to rapid and long-term clearing in patients with very severe scalp involvement who failed conventional topical and systemic treatment.

Sleep apnea as a comorbidity in obese psoriasis patients: a cross-sectional study. Do psoriasis characteristics and metabolic parameters play a role?

BACKGROUND: Psoriasis is associated with a variety of comorbidities such as obesity and cardiovascular disease. OBJECTIVE: In a cross-sectional study, we explored whether obstructive sleep apnea and hypopnea syndrome (OSAHS) is associated with psoriasis characteristics and metabolic parameters. METHODS: Thirty-five patients with chronic plaque psoriasis underwent a nocturnal polysomnography study and were analysed for Apnoea-Hypopnoea Index to assess OSAHS severity and Framigham score to predict the absolute risk of coronary artery disease at 10 years. The association of OSAHS with psoriasis was examined according to psoriasis characteristics (PASI and DLQI scores, disease duration and previous use of systemic treatments), metabolic parameters (Body Mass Index - BMI, waist to hip ratio - WHR, lipid profile) and other comorbidities (obesity, hypertension, arthritis and cardiovascular disease). RESULTS: There was no correlation between psoriasis characteristics and OSAHS. Psoriasis patients with OSAHS presented more frequent snoring and lower sleep quality compared with those without OSAHS. In univariate analyses, OSAHS was associated with increased BMI and hypertension in psoriasis patients. In multivariable logistic regression models, there was statistically significant evidence that only BMI and hypertension were associated with increased risk of OSAHS, adjusting for psoriasis characteristics, age and gender. Presence of metabolic syndrome, WHR, and smoking were not significant risk factors for OSAHS. In subgroup analyses, OSAHS correlated with duration of psoriasis (>8 years) in women (P = 0.021) and with Framigham score in men (P = 0.035). CONCLUSION: OSAHS may be a comorbidity in obese psoriasis patients with hypertension. Treatment with continuous positive airway pressure and weight loss interventions should be initiated.

Prevalence of non classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency in Greek women with acne: a hospital-based cross-sectional study.

AIM: To determine the prevalence and frequency of non classical congenital adrenal hyperplasia (NC-CAH) due to 21-OHD at the time of clinical presentation and at the peripubertal period in a substantial sample of Greek women with acne and to investigate the correlation of serum T, 17-OHP and DHEA-S with acne appearance at the time of clinical presentation. METHODS: One hundred and twenty-three unselected women with hyperandrogenemic symptoms were examined. After the ACTH stimulation test, 6 (4.9%) women were diagnosed with NC-CAH due to 21-OHD. RESULTS: There was not any statistical significant difference in the frequency of peripubertal acne between NC-CAH group of patients (6.4%) and patients with hyperandrogenemia of other aetiology (93%), mainly ovarian (P = 0.41). However, there was a statistical significant difference in the prevalence of acne at the time of clinical examination between the two groups (P = 0.04). Acne was present in 83.3% of women with NC-CAH vs. 41.02% of women in the hyperandrogenic group without NC-CAH. A statistically significant decrease of acne from the peripubertal time to the time of clinical examination in the group of women with hyperandrogenemia of other aetiology (-21.37%) was observed compared to women with NC-CAH (P < 0.001). CONCLUSION: We have shown that acne persists from peripubertal period to adult life in NC-CAH women whereas it tends to diminish in women with hyperandrogenemia of other aetiology. Acne is a prominent finding in women with NC-CAH. Serum concentrations of 17-OHP after ACTH stimulation (17-OHP6O ) should be investigated in women with persistent acne in adult life.

Adipocyte factors, high-sensitive C-reactive protein levels and lipoxidative stress products in overweight postmenopausal women with normal and impaired OGTT.

OBJECTIVE: In obese postmenopausal women we assessed leptin and adiponectin, high-sensitive C-reactive protein (hsCRP), serum lipids and lipoxidative stress products: oxidized LDL (oxLDL) and malondialdehyde (MDA), in relation to impaired glucose tolerance (IGT). METHODS: Thirty-eight overweight/obese postmenopausal women were included in the study. Eighteen with normal glucose metabolism (NGT) and twenty with IGT, as it is diagnosed by OGTT. Serum leptin, adiponectin, hsCRP and MDA were measured at time 0 and 120 min of OGTT while total-cholesterol, LDL, HDL, triglycerides, oxLDL and anti-oxLDL autoantibodies at time 0. Insulin resistance (HOMA)/sensitivity (QUICKI) indexes were estimated. RESULTS: In subjects with NGT, hsCRP was positively correlated with fasting leptin and HOMA, while in subjects with IGT negatively with QUICKI. In both groups, hsCRP was positively correlated with fasting insulin, body mass index and waist circumference. Fasting adiponectin was positively associated with HDL in both groups and negatively with triglycerides in subjects with NGT as well as with serum glucose levels at time 120 min of OGTT in subjects with IGT. No association was observed between oxLDL and adipokines. A significant positive association was found between oxLDL and HOMA in subjects with IGT. During OGTT there was a significant increase of leptin and MDA levels in both groups. CONCLUSIONS: A relationship exists between obesity, insulin and sub-clinical inflammation. Leptin and lipid peroxidation are linked to hyperglycaemic state while oxLDL might be considered as a predictor of insulin resistance. Adiponectin could exert its antiatherogenic effect through HDL independently of the presence of IGT.

Circulating oxidized LDL levels, current smoking and obesity in postmenopausal women.

OBJECTIVE: The aim of the present study was to estimate circulating oxidized low-density lipoprotein (oxLDL) levels in postmenopausal women and evaluate their association with obesity and smoking status. DESIGN AND METHODS: The study included 135 postmenopausal women aged 52-75 years. Forty of them were overweight (BMI 32.4+/-6.4) and non-smokers (Group A), 40 non-overweight (BMI 22.6+/-1.8) and smokers (Group B) and 55 non-overweight (BMI 23.5+/-1.4) and non-smokers (Group C). oxLDL and antibodies against them (anti-oxLDL) were measured using ELISA. Serum total cholesterol, LDL, HDL and triglycerides were measured in an automated analyzer. RESULTS: Total cholesterol, LDL, HDL and oxLDL serum levels were significantly elevated in Group A as compared to Group B or C, as well as oxLDL in Group B in comparison to Group C (p<0.001). Triglycerides and anti-oxLDL were increased in Group A in comparison to Group C (p=0.043 and 0.023). Total cholesterol, LDL, triglycerides and anti-oxLDL did not differ between Groups B and C, while HDL was decreased in Group B as compared to Group C (p<0.001). A significant positive correlation was found between oxLDL and LDL in Group A (r=0.53, p<0.001) as well as in Group C (r=0.955, p

Cutaneous manifestations in relation to immunologic parameters in a cohort of primary myelodysplastic syndrome patients.

BACKGROUND: Cutaneous lesions in myelodysplastic syndrome (MDS) may be specific or not and may reveal bone marrow transformation. Our purpose was to investigate in a cohort of 84 MDS patients the correlation of cutaneous findings with immunologic parameters and prognostic features of MDS in order to clarify their potential clinical significance. MATERIALS AND METHODS: We studied a cohort of 84 newly diagnosed MDS patients in order to assess the cutaneous findings present at the time of diagnosis and during 1 to 3 years of follow-up. We described the clinical variety of cutaneous findings ascertained by histology. We also looked for any association between the group of MDS patients with skin manifestations and MDS subtype, immunologic and prognostic features highlighting transformation to acute leukaemia. RESULTS: Twenty-one patients presented cutaneous manifestations: 1 patient developed leukaemia cutis, 6 patients photosensitivity not associated with autoimmune disease, 3 prurigo nodularis, 2 Sweet's syndrome, 6 leucocytoclastic vasculitis, 2 ecchymoses and purpura associated with preexisting relapsing polychondritis, 1 patient subcutaneous nodules associated with Wegener's granulomatosis and 1 patient with malar rash and oral ulcers associated with preexisting systemic lupus erythematosus. Adjusted for age and gender, the presence of skin findings constitutes a significant predictor of the high-risk MDS subgroup (odds ratio, 3.59; 95% confidence interval, 1.18-10.92). Hypergammaglobulinemia was significantly higher in the MDS subgroup with skin manifestations (P = 0.03). CONCLUSION: Most MDS patients with cutaneous manifestations belong to the high-risk MDS subgroup and present hypergammaglobulinemia. Early biopsy of skin lesions in myelodysplasia is indicated.

Home and leisure activities and childhood knee injuries.

AIM: To assess the relative occurrence of non motor-vehicle knee injuries and identify important clusters that can be targeted for preventive interventions. METHODS: The study subjects covered 2167 children (0-14 years) who suffered non motor-vehicle knee injuries out of 66870 registered during a three-year period in an established Emergency Department Injury Surveillance System (EDISS). A more serious joint injury was identified in 263 (12%) children, whereas the remaining 1904 children had only soft tissue knee injuries. RESULTS: The incidence of non motor-vehicle knee injuries was estimated at 6.5 per 1000 children-years. Both the incidence of knee injuries and the male-to-female ratio increase with increasing age, reflecting the gender and age pattern of physical activity. Three clusters were identified: The first consisted of more serious knee injuries among older children, frequently resulting after a fall from stairs or a collision in school during winter months; the second cluster consisted of rather minor knee injuries occurring mostly among younger girls at home or in playgrounds, following a fall after stumbling or hit by an object while playing, especially during the summer; the third cluster comprised injuries among older boys, sustained mainly subsequent to overexertion in a sports area. CONCLUSION: Knee injuries tend to be more common among boys but more serious among girls. More and less serious knee injuries tend to fall into distinct clusters that could facilitate prioritization of preventive measures.