Sociodemographic, clinical, laboratory, treatment and prognostic characteristics of 156 generalized pustular psoriasis patients in Turkey: a multicentre case series.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe inflammatory disease characterized by widespread and superficial sterile pustules on an erythematous background. OBJECTIVES: This multicentre study aimed to determine the clinical profile and course in a large cohort of patients with GPP. METHODS: One hundred and fifty-six GPP patients (mean age, 44.2 ± 18.7 years) who met the diagnostic criteria of the European Consensus Report of GPP were included in the study. Sociodemographic characteristics, quality of life, triggering factors of the disease, clinical, laboratory, treatment and prognostic features were evaluated. RESULTS: 61.5% of the patients were female. The rate of working at or below the minimum wage (≤$332.5/month) was 44.9%. Drugs (36.5%) were the most common trigger. While hypocalcaemia (35.7%) was the most important cause of GPP during pregnancy, systemic steroid withdrawal (20%) was the most frequently reported trigger for infantile/juvenile and mixed-type GPP (15%) (P < 0.05). Acute GPP (53.8%) was the most common clinic. Nails were affected in 43.6% of patients, and subungual yellow spots (28.2%) were the most common change. In annular GPP, fever (P < 0.001) and relapse frequency (P = 0.006) were lower than other subtypes, and the number of hospitalizations (P = 0.002) was lower than acute GPP. GPP appeared at a later age in those with a history of psoriasis (P = 0.045). DLQI score (P = 0.049) and joint involvement (P = 0.016) were also higher in this group. Infantile/juvenile GPP was observed in 16.02% of all patients, and arthritis was lower in this group (24.4 vs. 16%). GPP of pregnancy had the worst prognosis due to abortion observed in three patients. CONCLUSIONS: Recent advances in treatment have improved mortality associated with GPP, but abortion remains a significant complication. Although TNF-α inhibitors have proven efficacy in GPP, they can also trigger the disease. Mixed-type GPP is more similar to acute GPP than annular GPP with systemic manifestations and course.

Clinical and demographic features of hidradenitis suppurativa: a multicentre study of 1221 patients with an analysis of risk factors associated with disease severity.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. AIM: In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. METHODS: In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. RESULTS: The mean age of disease onset was 26.2 ± 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 ± 8.4 years with a delay in diagnosis of 5.8 ± 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P < 0.05 for all). Acne (40.8%), pilonidal sinus (23.6%) and diabetes mellitus (12.6%) were the most prevalent associated diseases. Of the various therapies used, antibiotics (76.4%) were most common followed by retinoids (41.7%), surgical interventions (32.0%) and biologic agents (15.4%). Logistic regression analysis revealed that the most important determinants of disease severity were male sex (OR = 2.21) and involvement of the genitals (OR = 3.39) and inguinal region (OR = 2.25). More severe disease was associated with comorbidity, longer disease duration, longer diagnosis delay and a higher number of smoking pack-years. CONCLUSIONS: Our nationwide cohort study found demographic and clinical variation in HS, which may help broaden the understanding of HS and factors associated with disease severity.

Behcet disease and pregnancy.

PURPOSE: Behcet disease (BD) is a multisystemic vaculitis commonly diagnosed in reproductive years. The authors aimed to investigate the relationship between BD and pregnancy outcomes. MATERIALS AND METHODS: In this multicenter retrospective survey study, the authors compared the pregnancy outcomes of BD patients with the healthy controls. RESULTS: A total of 298 pregnancies of 94 patients with BD and 219 pregnancies of 95 healthy controls were evaluated. The mean birth weight of all babies of women with BD and the control group were 3,214 grams and 3,351 grams, respectively (p = 0.028). The miscarriage rates were also higher in the BD group. The com- plication rates of pregnancy with hypertension, preeclampsia, preterm labour in the study group and the control group were 12.8% and 11.6%, respectively (p = 0.489). CONCLUSION: The current study demonstrated that BD patients delivered smaller babies and they have higher miscarriage rates when compared to the healthy controls which might be due to the vasculitis of the placenta.

Gamma glutamyl transferase activity: relationship with thoracic aortic intima media thickness and inflammation.

OBJECTIVE: Increased serum gamma-glutamyl transferase (GGT) activity is known to be associated with atherosclerotic diseases. Thoracic aortic intima-media thickness (IMT) was reported as a marker of preclinical atherosclerosis. However, there is a lack of research directly examining the relationship between serum GGT activity and thoracic aortic IMT. Therefore, we aimed to investigate the association between serum GGT activity and thoracic aortic IMT. PATIENTS AND METHODS: The study population consisted of 329 patients without coronary artery disease, who underwent transesophageal echocardiography (TEE) examination for various indications from January 2011 to April 2013. GGT, high-sensitivity C-reactive protein (hs-CRP) and other biochemical markers were measured in all patients. The patients were classified into tertiles according to their GGT activities (GGTlow < 19 U/l, GGTmid ≥ 19 U/l < 29 U/l, and GGThigh ≥ 29). RESULTS: The highest aortic IMT values were observed in the GGThigh group compared with the GGTmid and GGTlow groups (p < 0.05, for all). Also, aortic IMT values in the GGTmid group were higher than in the GGTlow group (p < 0.05). Multivariate regression analysis showed that GGT activity was independently associated with aortic IMT (ß = 0.487, p < 0.001) hs-CRP (ß = 0.282, p < 0.001), and triglyceride level (ß = 0.161, p = 0.007). CONCLUSION: The higher serum GGT concentrations within the "normal" range were associated with a greater IMT of the thoracic aorta. GGT activity may be a predictor of the extent of subclinical aortic atherosclerosis assessed with thoracic aortic IMT.

The prognostic role of the new entity butterfly-like facial rash and cutaneous findings in patients with crimean congo haemorrhagic fever.

BACKGROUND: Crimean Congo Haemorrhagic Fever (CCHF) is a deadly fever caused by Nairovirus (family Bunyaviridae). It is an important public health problem in hyperendemic regions with a high mortality rate. There have been many studies on cutaneous findings in other viral infections, however, our knowledge is limited regarding CCHF. The aim of this study was to evaluate the cutaneous manifestations of CCHF and their relationship with mortality. METHODS: Patients who were diagnosed CCHF between April 1 and September 1, 2011 were enrolled in this study. Dermatologic examination and laboratory analysis were obtained during hospitalization. RESULTS: A new entity that is first reported in this study as a butterfly-like facial rash was recognized in 76 patients (42.9%). It was not correlated with mortality but could coexist with other dermatological signs of CCHF. The major outcome was the increased mortality rate with other cutaneous findings (especially ecchymosis, purpura and petechia) (P < 0.01). While 12 deceased individuals had one or more skin findings, only one patient died without any cutaneous manifestations. Mortality rate was higher in patients with purpura than the patients with pethesia. The statistically significant correlation was observed between cutaneous manifestations and elevation of aspartate aminotransferase (AST) levels (P = 0.009). Platelet levels were lower in patients with cutaneous involvement (P < 0.01). No statistically significant relationship was found between cutaneous findings and alanine aminotransferase (ALT), white blood cell (WBC), Haemoglobin (Hb), creatinine kinase (CK) and lactate dehydrogenase (LDH) levels. LIMITATIONS: Histopathologic examination of facial rash could not be done because of haemostatic failure. CONCLUSION: The results of this study confirm that cutaneous findings can be a clue regarding the prognosis of the patients with CCHF. Physicians should be aware that CCHF may present with a butterfly-like rash on the face.

Crimean-Congo haemorrhagic fever: peritoneal and pleural effusion.

In Crimean-Congo haemorrhagic fever (CCHF), haemorrhagic manifestations are usually petechiae and ecchymoses on mucous membranes and skin. Rarely, there is bleeding from the nose, gingiva, gastro-intestinal tract, genito-urinary tract, brain and lungs. A 13-year-old boy with CCHF presented with gastro-intestinal bleeding and developed peritoneal and pleural effusion. He made a complete recovery with supportive treatment and ribavirin, without requiring chest or peritoneal fluid drainage. To our knowledge, this is the first report of CCHF associated with peritoneal and pleural fluid.

Aortic elastic properties are impaired in patients with Graves' ophtalmopathy.

BACKGROUND: Aortic elastic properties have been shown to be an important predictor of cardiovascular morbidity and mortality. Hyperthyroidism was shown to be an important cause of impaired aortic elastic properties both due to the direct effect of the thyroid hormones on the aorta and also due to modulating effects of thyroid hormones on the vascular renin angiotensin system. However, there is no study investigating the aortic elastic properties in Graves's patients who were euthyroid. The goal of the present study was to investigate the aortic elastic properties of patients with Graves' Ophtalmopathy (GO) who had been euthyroid for at least 3 months. PATIENTS AND METHODS: A cross-sectional study was performed on 47 GO patients and 27 controls. Aortic-diastolic and aortic-systolic diameters, aortic strain, aortic distensibility, and aortic stiffness indices were calculated from the diameter of the thoracic aorta as measured by transthoracic echocardiography. RESULTS: The aortic stiffness index was markedly increased (31 ± 26 vs. 17 ± 8.9; p = 0.015) and aortic strain was markedly reduced (20.3 ± 10 % vs. 25.9 ± 12 %; p = 0.046) in the GO group relative to the control group. Aortic distensibility was statistically significantly decreased in the GO group as compared to the control group (9.5 ± 5.7 10(-3)/kPa vs. 13.5 ± 7.1 10(-3)/kPa; p = 0.022). Weak correlations were detected between GO severity and aortic distensibility (r = -0.333, p = 0.011) as well as the aortic stiffness index (r = 0.266, p = 0.044). CONCLUSIONS: Aortic elastic properties were impaired in patients with GO. Therefore, patients with GO, especially those with severe manifestations of the condition, should be followed closely with regard to the occurrence of future cardiovascular events.

A histopathological approach: when papulopustular lesions should be in the diagnostic criteria of Behçet's disease?

UNLABELLED: BACKGROUNDPapulopustular lesions (PPL) are commonly seen cutaneous lesions in Behçet's disease (BD). Some authorities do not recommend using cutaneous lesions for diagnostic criteria because these lesions are confused with acne vulgaris. Objective To understand if PPL have diagnostic value in BD. METHODS: Biopsy was performed on papulopustular lesions of Behçet's patients for histopathological examination to compare with the control group composed of patients with acne vulgaris. Forty two patients with BD and 21 control patients with acne vulgaris were enrolled in the study. Histopathological findings were classified as leukocytoclastic vasculitis, lymphocytic vasculitis, superficial and/or deep perivascular infiltration, and folliculitis and/or perifolliculitis. RESULTS: Of the specimens from 42 Behçet's disease patients, 7 (16.7%) revealed leukocytoclastic vasculitis and 3 (7.1%) had lymphocytic vasculitis, 15 (35.7%) had superficial perivascular and/or interstitial infiltration, 12 (28.6%) had superficial and deep perivascular and/or interstitial infiltration, 5 (11.9%) had folliculitis or perifolliculitis. Of the control group patients, 9 (42.9%) had folliculitis or perifolliculitis, 8 (38.1%) revealed superficial perivascular inflammation and 4 (19.0%) revealed mixed superficial and deep perivascular inflammation. Vasculitis was not encountered in any control group specimens. Vasculitis ratio was statistically higher in Behcet's disease patients (P = 0.016). CONCLUSIONS: We evaluated more patients than previous related studies reported in the literature. Histopathological findings of vasculitis were encountered only in BD group. Non-vasculitis histopathological findings were also encountered in the control group patients. In conclusion, PPL can be used as a diagnostic criterion of BD subsequent to finding vasculitis in histopathological examination of the biopsy specimen of the PPL.

Serum zinc and alkaline phosphatase values in pediatric bone marrow transplantation patients.

Zinc (Zn) plays an important role in the maintenance of immune functions, including cellular/humoral immunity, and in the prevention of oxidative injury. Therefore, the maintenance of a normal Zn status may be important in bone marrow transplantation (BMT) patients. Serum Zn levels were determined in 35 children during the BMT period. In addition, as Zn-related factors, serum Cu levels and alkaline phosphatase (AP) activity were also measured. There was a significant decrease in Zn and AP values during the immediate post-transplant period (lowest at day +7) when compared to pre-BMT levels (p <.01). The patients who developed hypozincemia were more likely to be transplanted for a diagnosis of malignant disorder and were younger, and adverse events appeared to occur more frequently. This preliminary study suggests that maintaining a normal Zn status may be important in BMT patients and that Zn deficiency may be a risk factor for adverse events.