Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients.

BACKGROUND: Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. AIM: To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. METHODS: We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. RESULTS: In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 ± 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). CONCLUSION: Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Toll-like receptor 9 polymorphism in patients with erythema multiforme, Stevens Johnson syndrome and Stevens Johnson syndrome/toxic epidermal necrolysis overlap syndrome.

BACKGROUND: "Toll like receptor" (TLR) 9 functions in stepping in of native immune system against different viral and bacterial pathogens and induction of adaptive immune response effectively. TLR 9 gene polymorphism makes host predisposed to microbial pathogens by affecting thefunctional capabilities of the receptor. OBJECTIVE: We aimed to determine if TLR 9 gene polymorphism makes a predisposition to "erythema multiforme" (EM), "Stevens Johnson syndrome" (SJS) and "Stevens Johnson syndrome/toxic epidermal necrolysis overlap syndrome" (SJS/TEN). METHODS: Forty-two patients clinically and/or histopathologically diagnosed as EM, SJS, and SJS/TEN overlap syndrome and 50 healthy control subjects were enrolled in our study. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method was applied for TLR 9 gene 1237 thymine/cytosine (T/C) polymorphism. Genotypes were determined according to bands occurring on agarose gel electrophoresis. RESULTS: In patients group, the frequencies of TT and TC genotypes were 73.8% and 26.2% while CC genotype wasn't detected. In control group, the frequencies of TT, TC and CC genotypes were 74%, 24%, and 2%. There wasn't a statistically significant difference for TT, TC and CC genotypes between patients and controls. The frequencies of T and C alleles were 84.5% and 15.5% in patients and 86% and 14% in controls, respectively. CONCLUSION: Our results showed that there isn't any association between TLR gene polymorphism and EM, SJS, SJS/TEN overlap syndrome (Tab. 1, Fig. 1, Ref. 30).

Vitiligo and ocular findings: a study on possible associations.

OBJECTIVE: In this study, we aimed to evaluate the ocular findings in vitiligo patients and reveal any clinical feature that might suggest an association or a risk factor. BACKGROUND: Very few reports in the literature are available about the ocular findings in vitiligo and the possible associations of the ocular findings in vitiligo patients have not been studied so far. METHODS: A total of 45 patients with previously documented cutaneous vitiligo were examined for ocular findings. Demographic features including age, gender, duration of vitiligo, presence of associated autoimmune diseases, type of vitiligo and the anatomical distributions of vitiligo were recorded to evaluate a possible relationship with the ocular findings. Univariate and multivariate analyses as well as cluster analysis were performed. After description of the clusters, the Mann-Whitney U-test and Fisher's exact test were used to determine the variables. Concordance among the variables in each group was evaluated with the McNemar test. RESULTS: Ten patients had ocular findings that included anterior segment (iris) involvement, ring-like peripapillary atrophy around the optic nerve, atrophy of pigment epithelium, focal hypopigmented spots and diffuse hypopigmentation. The presence of periorbital vitiligo was significantly related to the ocular findings. Cluster analysis revealed concordances between periorbital and genitalial localizations of vitiligo and ocular findings. CONCLUSION: The number of patients and the range of ocular findings in our study are insufficient to make definite conclusions but anatomical localizations, primarily periorbital and to a lesser extent genitalial vitiligo, seem to be the most probably alerting features for ocular findings.

Successful labor in the course of chronic lymphocytic leukemia (CLL) and management of CLL during pregnancy with leukapheresis.

We describe the successful management of a 30-year-old woman in the second trimester of her pregnancy with chronic lymphocytic leukemia (CLL) in stage IV by using only leukapheresis. We applied three sessions (courses) of leukapheresis throughout the pregnancy. The procedure did not have any significant adverse effect on the patient and the fetus. The patient gave birth vaginally to a healthy boy, weighing 3100 g, at 39 weeks of gestation. Seven months after delivery, Richter's syndrome developed in the patient. We conclude that leukapheresis may provide an alternative for palliative treatment to chemotherapy in pregnant patients with CLL. To our knowledge, this is the fourth reported case of CLL in pregnancy, and the first management of CLL during pregnancy with leukapheresis.

Echocardiographic findings in subjects with psoriatic arthropathy.

BACKGROUND: Psoriatic arthropathy (PA) is a seronegative arthropathy with a 5-20% prevalence among psoriatics. In recent years, cardiovascular abnormalities have been shown in patients with seronegative arthropathies. OBJECTIVE/AIM: Since echocardiography is a non-invasive method to evidence cardiac abnormalities, we planned a study to evaluate heart involvement in subjects with psoriatic athropathy using this method. METHODS: A total of 21 subjects (15 women, six men) aged from 34 to 71 years were involved in this study. After PA diagnosis was confirmed by skeletal scintigraphic survey, patients were evaluated by Doppler echocardiogram for cardiovascular disturbances and the results were compared with those for a sex- and age-matched control group. RESULTS: The left ventricle end-diastolic and end-systolic diameters of the PA group were statistically different from those of the control group (P < 0.05), but no difference was observed in ejection fraction and the mitral E/A ratios. The presence of diastolic dysfunction was significantly related to the presence of arthropathy and the duration of psoriasis (P < 0.05). CONCLUSION: We conclude that mild diastolic dysfunction may accompany PA but our data should be confirmed by further studies.

Idiopathic thrombocytopenic purpura in pregnancy: a single institutional experience with maternal and neonatal outcomes.

We observed 13 pregnant women of 70 females with idiopathic thrombocytopenic purpura (ITP) from January 1992 through September 2002. Thirteen mothers with ITP gave birth to twelve babies and two fetuses died. One of the pregnancies produced twins. Seven of the cases were diagnosed with ITP before pregnancy and six during pregnancy. One of the thirteen pregnancies was complicated by preeclampsia, one by ablatio placentae, and one by intrauterine death. Seven mothers received corticosteroid treatment, four high-dose immunoglobulin therapies, and one underwent splenectomy in the second trimester of gestation. At the time of delivery six mothers had normal platelet counts and seven had low platelet counts. Nine deliveries were by vaginal route and four were by cesarean section. Eleven infants were born with normal platelet counts and one was thrombocytopenic at the time of delivery. No infant showed any clinical signs of hemorrhage and there were no neonatal complications. Two fetuses died; one of them because of ablatio placentae and the other was intrauterine dead. In conclusion, ITP in pregnancy requires the management of two patients, the mother and her baby; hence, the close collaboration of a multidisciplinary group composed of a hematologist, obstetrician, anesthesiologist, and neonatologist is essential.