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.

Breast cancer subtypes can be a predictor of pathologic complete response and survival in the neoadjuvant setting for T4 noninflammatory breast cancer.

BACKGROUND: The aim of this study is to identify whether the breast cancer subtypes are predictors of pathologic complete response (pCR) to neoadjuvant chemotherapy (NAC) and survival in patients with T4 noninflammatory breast cancer. METHODS: The records of 181 patients treated with anthracycline +/- taxane based NAC followed by mastectomy and radiation therapy +/- hormonotherapy were evaluated. The role of intrinsic subtypes of the tumor including luminal A, luminal B, HER2, and triple-negative on pCR and survival were analyzed. RESULTS: The median follow-up was 44 months (range: 16-82 months). All patients received a median four cycles of NAC. Twenty-three patients (12.7%) were found to have pCR. In the univariate analysis, the intrinsic subtypes of the tumor had significant effect on pCR (p < 0.01). Also, intrinsic subtypes were significant predictors of pCR to NAC in the multivariate analysis (p < 0.01; hazard ratio, 2.4; 95% confidence interval, 1.1-6.8). While patients with triple-negative tumors had the highest rate of pCR (29%), this rate was the lowest in patients with HER2 tumors (4.2%). Five-year DFS was also significantly lower in patients with triple-negative (24%) and HER2 (21%) tumors compared to luminal A (61%) subtype (p < 0.0001). Likewise, 5-year OS was poorer in patients with triple-negative tumors (30%) and HER2 tumors (%31) compared to both luminal A (70%) and luminal B (68%) subtypes (p < 0.0001). CONCLUSIONS: It can be concluded that breast cancer subtyping defines the extent of response to NAC and has a significant effect on survival in patients with T4 noninflammatory breast cancer.

The value of spectrophotometric intracutaneous analysis in the noninvasive diagnosis of nonmelanoma skin cancers.

BACKGROUND: Spectrophotometric intracutaneous analysis (SIAscopy) is a recently introduced, noninvasive, rapid and practical method for monitoring pigmented lesions, which calculates the amount of collagen, melanin and haemoglobin deep in the papillary dermis. AIM: To evaluate the value of SIAscopy in the diagnosis of nonmelanoma skin cancers (NMSC). METHODS: In total, 80 lesions of 76 patients were clinically evaluated by the first investigator, and the data recorded. Eight months after the clinical evaluation, all lesions were evaluated again by the same investigator, using images (SIAgraphs) obtained by the SIAscope. All SIAgraphs were also evaluated by a second investigator, and all dermatoscopic images by a third, independently of each other. All diagnoses were compared with histopathological diagnoses. RESULTS: The clinical diagnosis was calculated to have a sensitivity of 79% and specificity of 84%. The SIAscopic diagnoses of the first and second investigators had a sensitivity of 55% and 93%, and a specificity of 88% and 53%, respectively, while the dermatoscopic diagnoses of the third investigator had a sensitivity of 86% and specificity of 80%. There was no statistical accordance between the first and second investigators according to the accuracy of SIAscopic diagnoses (P < 0.01). The area under the curve for the receiver operator characteristic was 0.82 for clinical diagnosis, 0.73 and 0.80 for the SIAscopic evaluation of the first and the second investigators, respectively, and 0.87 for the dermatoscopic evaluation of the third investigator. CONCLUSIONS: Our findings show that dermatoscopic findings are more valuable than SIAscopic and clinical findings for the noninvasive diagnosis of NMSC. We consider that SIAscopy makes no substantial contribution towards the differential diagnosis of NMSC.

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).

Investigation of ABCB1 gene polymorphism with colchicine response in Behçet's disease.

Colchicine is commonly used in the treatment of Behçet's disease. However, some patients are unresponsive to colchicine treatment. Adenosine triphosphate-binding cassette subfamily B member 1 (ABCB1) transports colchicine out of cells. We investigated a possible association of C3435T polymorphism of the ABCB1 (MDR1) gene with colchicine response in patients with Behçet's disease. We randomly selected 97 patients with Behçet's disease, examined ABCB1 (MDR1) gene C3435T polymorphisms, and evaluated patient responses to colchicine. Forty-three patients were colchicine responsive, while the remaining 54 patients were unresponsive. No significant difference was found between genotypic and allelic frequencies of the ABCB1 C3435T polymorphisms in patients with Behçet's disease and healthy volunteers. Also, there was no significant difference among responsive and nonresponsive patients. We concluded that ABCB1 C3435T polymorphism is not associated with a colchicine response in patients with Behçet's disease.

Effect of levothyroxine treatment on clinical symptoms and serum cytokine levels in euthyroid patients with chronic idiopathic urticaria and thyroid autoimmunity.

BACKGROUND: Screening for thyroid autoimmunity in patients with chronic idiopathic urticaria (CIU) is generally recommended. However, there are not yet sufficient data as to whether levothyroxine treatment is beneficial for the clinical symptoms of CIU in patients with thyroid autoimmunity. AIM: We investigated the effect of levothyroxine treatment on clinical symptoms and serum tumour necrosis factor (TNF)-alpha, interleukin (IL)-10 and interferon (IFN)-gamma levels in euthyroid patients with CIU and thyroid autoimmunity. METHODS: In total, 15 patients with CIU and positive thyroid autoantibodies were randomized to receive either levothyroxine plus 5 mg/day desloratadine (suppression group, n = 8) or 5 mg/day desloratadine alone (control group, n = 7) for 12 weeks. Clinical symptoms of CIU, thyroid hormone levels, thyroid antibodies and serum cytokine levels were assessed at baseline and after the treatment. RESULTS: There were significant improvements in pruritus score and severity of weals in both groups compared with baseline values, but when the two groups were compared, there was no significant difference in the patients' clinical symptoms. Thyroid antibody titres were not different according to intragroup and intergroup analysis. In the suppression group, serum IFN-gamma and TNF-alpha levels were increased after treatment with levothyroxine compared with baseline values and there was a borderline statistical significance (P = 0.05 for both). CONCLUSIONS: These results suggest that levothyroxine treatment is not a reasonable option in euthyroid patients with CIU and thyroid autoimmunity. Augmentation of cytokine production after levothyroxine treatment seems to be related to the immunomodulatory effects of TSH-suppressive treatment.

Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris.

BACKGROUND: Pemphigus is a chronic immunobullous disease, characterized by formation of blisters and erosions in skin and/or mucous membranes. This severe disease requires systemic immunosuppressive therapy. However, some patients are refractory, and long-term use of immunosuppressive agents can cause serious side-effects. Mycophenolic acid is increasingly being used as a corticosteroid-sparing agent or as monotherapy in immunosuppressive regimens. OBJECTIVES: We aimed to evaluate the effectiveness of mycophenolate sodium, a sodium salt of mycophenolic acid, in the treatment of pemphigus vulgaris. METHODS: Six patients who were diagnosed as pemphigus vulgaris with active, refractory disease were treated with mycophenolate sodium. Three patients received mycophenolate sodium monotherapy; three patients received mycophenolate in combination with steroid. All patients were monitored regularly. RESULTS: Mycophenolate sodium was well tolerated with a similar efficacy of mycophenolate mofetil, and no side-effects have been observed. CONCLUSIONS: Mycophenolate sodium appears to be an effective and safe alternative in the treatment of pemphigus vulgaris.

Detection of parvovirus B19 DNA in the lesional skin of patients with Behçet's disease.

BACKGROUND: There is disagreement in the current evidence for viral aetiologies in the pathogenesis of Behçet's disease (BD). OBJECTIVES: To investigate the presence of B19 DNA in skin lesions of patients with BD, compare with the skin of healthy controls and evaluate its role in the pathogenesis. METHODS: In total, 40 patients diagnosed with BD according to the criteria proposed by the International Study Group for Behçet's Disease and routinely followed up at our centre were enrolled into the study. All the patients selected were in the active phase of disease. Skin and blood samples of patients with BD and of the healthy volunteers were examined for B19 serology, histopathology and genome expression. RESULTS: The quantity of B19 DNA in nonulcerative BD lesions of was significantly different from ulcerative lesions in the study group and from the skin of the healthy controls (P < 0.01). For the nonulcerative lesions, real-time PCR analysis for B19 DNA was found to be 64% sensitive (95% CI 42.5-82.0) and 85% specific (95% CI 62.1-96.6) with a cut-off value of > 154 IU/mL (P < 0.001). CONCLUSIONS: To the best of our knowledge, this is the first study that provides evidence for a possible causal link between BD and parvovirus B19, and our data suggest the presence of the virus, particularly in intact, nonulcerative skin lesions of BD. Limitations to this study include the limited number of participants, and the fact that the exact source of B19 DNA was undetected.

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.

Comparison of short- and long-term cyclosporine A therapy in chronic idiopathic urticaria.

BACKGROUND: Chronic idiopathic urticaria is sometimes difficult to treat, with little response to antihistamine therapy. OBJECTIVE: In a limited number of severe chronic idiopathic urticaria patients, cyclosporine A (CsA) therapy was found to be effective. In this study, we compared the clinical efficacy and safety of short- and long-term CsA applications. METHODS: Twenty patients with severe disease, unresponsive to antihistamines and showing a positive autologous serum skin test (ASST) were randomized to 4 mg/kg per day of CsA for 4 or 12 weeks. The clinical efficacy was measured with a daily activity score of weal numbers and itch (UAS). The two groups were compared in terms of mean reduction in UAS and ASST response to pre-and post-treatment sera. RESULTS: The clinical improvement was dramatic in the first month of treatment in both groups. There was no significant difference in the frequency of responses, side effects and the reduction of UAS in either group. CONCLUSION: The preliminary results of our study suggest that CsA is clinically effective for chronic urticaria. The prolonged use of this therapy for more than 1 month provides little benefit in the clinical improvement.

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.

Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil.

Relapsing idiopathic nodular panniculitis is the term used to describe a group of diseases that presents as subcutaneous inflammatory nodules, fever and systemic symptoms and histopathologically displays inflammation within the fat lobules. There is no specific test for diagnosis and extensive investigations are required to exclude systemic causes of panniculitis. No uniform effective therapy is available and various drugs used include mainly corticosteroids alone or in combination with other immunosuppressive agents. Presented in this paper is an intractable case of idiopathic nodular panniculitis whose corticotherapy failed and could not be continued because of serious adverse effects. The rapid and good therapeutic response of the patient to mycophenolate mofetil monotherapy is discussed.