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 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.

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.

Internalized stigma in acne vulgaris and its relationship with quality of life, general health, body perception, and depression.

BACKROUND: Internalized stigma, adoption of negative attitudes, and societal stereotypes regarding acne vulgaris (AV) have not been previously studied. OBJECTIVE: To investigate the internalized stigma state in AV and determine its association with quality of life, perceived health, body image, and depression. METHODS: A total of 77 AV patients (43 female, 34 male; aged 19.7 ± 2.3 years) were enrolled in this cross-sectional study. The scales used in the study were Acne Internalized Stigma Scale (AISS), Acne Quality of Life scale (AQOL), FDA Global score, Perceived Health Status (PHS), Body Image Scale (BIS), and Beck Depression Inventory (BDI). RESULTS: Mean AISS scores (53.68 ± 13.6) were significantly higher in males than in females (57.41 ± 14.37, 50.39 ± 12.25, P = 0.042). There was a significant positive correlation between mean values of AISS and AQOL (r = 0.816, P < 0.001), FDA Global grade (r = 0.391, P = 0.002) and BDI (r = 0.440, P < 0.001). Lower PHS (P = 0.027) was another determinant of high AISS scores. The mean AISS score of patients with a family history was significantly lower than those without a family history (P = 0.007). VAS was also found to be correlated with mean values of AISS and AQOL. Linear regression analysis revealed that the most important determinant of internalized stigma was AQOL (ß = 0,632; P < 0.001), followed by gender (ß = -0,229; P = 0.001), FDA Global score (ß = 0,193; P = 0.007), and BDI (ß = 0,177; P = 0.024). DISCUSSION: Significant and independent predictive factors for high internalized stigma state were the negative quality of life, male gender, the severity of the illness, and depression. Therefore, internalized stigma may be one of the major factors responsible for the psychosocial burden of AV.

Oral health related quality of life and disease severity in autoimmune bullous diseases.

BACKGROUND: There is an increased risk of long-term dental and periodontal disease in autoimmune bullous diseases (AIBD). AIMS: In this cross-sectional study, we aimed to determine whether the oral health-related quality of life status (OHRQoL) was associated with disease severity and activity in patients with AIBD. SUBJECTS AND METHODS: 67 patients with AIBD were enrolled in this study. Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) was used to evaluate the disease severity. The score was categorized as a significant course (≥17) and moderate course (<17). Oral health impact profile-14 (OHIP-14) questionnaire was filled to assess the OHRQoL. Self-reported oral health status and oral lesion related pain score were also evaluated in the study group. RESULTS: OHIP-14 score was significantly higher in active patients (42.28 ± 13.66) than inactive patients (29.08 ± 12.25) (P = 0.004) and it was correlated with the pain score (6.33 ± 2.78; r = 0.409, P = 0.013). Furthermore, OHIP-14 score was higher in patients with a significant disease course (45.18 ± 15.08) (P = 0.010) than in patients with a moderate course (36.09 ± 9.73). CONCLUSIONS: OHRQoL may be useful in the disease management and treatment. Since it can be affected by both presence of oral erosions and disease severity, a collaboration between dermatologists and dentists could be crucial to the disease management in AIBD.

Unilateral nevoid telangiectasia accompanied by neurological disorders.

BACKGROUND: Unilateral nevoid telangiectasia (UNT) is a unique vascular dermatosis of ambiguous aetiology. OBJECTIVE: To investigate the role of neurological disorder in pathogenesis of the UNT. METHODS: We investigated eight consecutive patients with unilateral nevoid telangiectasia. Detailed dermatological and neurological examinations, and magnetic resonance imaging were performed on each patient. In case of presence of dysesthesia over the skin lesion, electroneuromyography was performed to determine any relationships between lesions and peripheral neuropathy. RESULTS: All the patients had hypoesthesia over the skin lesion. The cranial magnetic resonance imaging showed subcortical hamartomatous lesions in one patient and demyelinized plaques on the corpus of the caudate nucleus and the pontin area in another. Electroneuromyography evaluation was nonspecific. CONCLUSION: In our study, neurological disorders were associated with UNT. Thus, it can be speculated that neurological disorders might contribute to the development and/or progression of UNT. Patients with UNT should be encouraged for neurological investigation.

Evaluation of JPEG and JPEG2000 compression algorithms for dermatological images.

BACKGROUND: Some image compression methods are used to reduce the disc space needed for the image to store and transmit the image efficiently. JPEG is the most frequently used algorithm of compression in medical systems. JPEG compression can be performed at various qualities. There are many other compression algorithms; among these, JPEG2000 is an appropriate candidate to be used in future. OBJECTIVE: To investigate perceived image quality of JPEG and JPEG2000 in 1 : 20, 1 : 30, 1 : 40 and 1 : 50 compression rates. METHODS: In total, photographs of 90 patients were taken in dermatology outpatient clinics. For each patient, a set which is composed of eight compressed images and one uncompressed image has been prepared. Images were shown to dermatologists on two separate 17-inch LCD monitors at the same time, with one as compressed image and the other as uncompressed image. Each dermatologist evaluated 720 image couples in total and defined whether there existed any difference between two images in terms of quality. If there was a difference, they reported the better one. Among four dermatologists, each evaluated 720 image couples in total. RESULTS: Quality rates for JPEG compressions 1 : 20, 1 : 30, 1 : 40 and 1 : 50 were 69%, 35%, 10% and 5% respectively. Quality rates for corresponding JPEG2000 compressions were 77%, 67%, 56% and 53% respectively. CONCLUSION: When JPEG and JPEG2000 algorithms were compared, it was observed that JPEG2000 algorithm was more successful than JPEG for all compression rates. However, loss of image quality is recognizable in some of images in all compression rates.

Recurrent bullous lesions associated with familial Mediterranean fever: a case report.

Familial Mediterranean fever (FMF) is an inherited, recurrent, inflammatory disease. Of its various cutaneous features, erysipelas-like erythema is the best known and most common skin lesion. We present a new case of FMF with recurrent bullous lesions. A 41-year-old woman was admitted to our clinic with tense bullae, 20 x 20 mm in diameter on the left shin. The patient had a history of fever, abdominal pain, peritonitis attacks and infertility. A lesional skin biopsy revealed subepidermal bullae and neutrophilic infiltration around dermal vessels. Direct immunofluorescence analysis was negative. Over the period of investigation, the lesion regressed spontaneously; 1 month later, a similar lesion appeared on the right wrist. Diagnosis of FMF was made according to the Tel-Hashomer criteria. Recognition of this peculiar skin lesion may lead to an earlier diagnosis of the disease.

Periodontal status in patients with pemphigus vulgaris.

OBJECTIVE: The aim of this study was to evaluate the periodontal status of pemphigus vulgaris (PV) patients and compare it with that of healthy controls. We also analysed the association between the periodontal condition and the clinical severity of the disease in PV patients. STUDY DESIGN: Twenty patients (nine women, 11 men; mean +/- s.d. age, 42.9+/-9.8 years) with PV and 22 healthy subjects (eight women, 14 men; mean +/- s.d. age, 40.5 +/- 12.1 years) were included in the study. The periodontal status of all subjects was evaluated according to the Community Periodontal Index of Treatment Needs (CPITN). PV patients were also assessed for Clinical Severity Score (CSS). RESULTS: The mean CPITN values were observed to be higher in PV patients (2.8 +/- 0.7) compared with those of healthy controls (1.0 +/- 0.8) (P < 0.001). Nevertheless, there was no statistically significant difference in CPITN values according to the CSS (P = 0.4). The number of carious teeth was significantly higher in PV patients than that in healthy subjects. CONCLUSIONS: Our results showed that periodontal status is worse in PV patients. Moreover, PV might contribute to the development and / or progression of periodontitis. PV patients should be encouraged for long-term periodontal follow up.

Progressive symmetrical erythrokeratoderma: report of a Turkish family and evaluation for loricrin and connexin gene mutations.

We report here the first Turkish patient with progressive symmetric erythrokeratoderma. The patient's skin lesions appeared in the axillae at 3 months of age, and gradually spread to other flexural areas and to the trunk. Dermatological examination of the boy at 3.5 years of age revealed symmetric, hyperkeratotic plaques with erythematous outlines on the neck, wrists, armpits, trunk and posterior knees. The histopathological changes were nonspecific, including marked hyperkeratosis, irregular acanthosis, focal papillomatosis and perivascular lymphocytic infiltrates. Molecular studies of the loricrin (LOR), connexin 31 (GJB3) and connexin 30.3 (GJB4) genes did not identify a disease-causing mutation. These results further underline the genetic heterogeneity of the erythrokeratodermas.

Relationship between periodontal findings and the TNF-alpha Gene 1031T/C polymorphism in Turkish patients with Behçet's disease.

BACKGROUND: Genetic factors that predispose individuals to Behçet's disease (BD) and periodontal disease. Tumour necrosis factor-alpha (TNF-alpha) has been implicated in the pathogenesis of both BD and periodontal disease. The relationship with periodontitis and the pathogenesis of BD has not yet been determined. OBJECTIVE: The aim of the present study was to investigate the possible relation of the periodontal scores and single nucleotide polymorphism of TNF-alpha-1031T/C with BD compared with healthy controls (HC) and recurrent aphtous stomatitis (RAS). We also sought to determine the effects of periodontal condition and TNF-alpha-1031T/C polymorphism on clinical severity of BD. METHODS: Eighty-two unrelated patients with BD, 42 RAS patients and 77 HC were enrolled in the study. Periodontal status of all subjects was evaluated according to the World Health Organization community periodontal index of treatment needs (CPITN). For genotyping, polymerase chain reaction-restriction fragment length polymorphism was employed. RESULTS: The mean CPITN was observed to be higher in patients with BD compared with HC and RAS (P < 0.001). TNF-alpha-1031C allele was significantly higher in patients with BD (P = 0.023) and RAS (P = 0.007) compared with HC. Mean CPITN was higher in CC genotype compared with other genotypes (P = 0.004). Moreover, CPITN and CC genotype was found to be correlated with the severity of the disease. CONCLUSIONS: Our data indicate that the TNF-alpha-1031T/C gene polymorphism (CC genotype) is a risk factor for periodontitis, RAS and BD patients and also suggests that long-term periodontal follow-up and education of oral hygiene in patients with BD may help to prevent the development and/or progression of the disease.

Cutaneous alternariosis in a patient with systemic lupus erythematosus.

Alternaria species are common saprophytic fungi that naturally subsist on decaying plant materials, and occasionally may cause diseases in human beings and domestic animals. They can be a potential opportunistic pathogen in immunocompromized hosts or those with significant underlying disease. However, rarely they are also pathogen in otherwise healthy hosts. We report here the first case of cutaneous alternariosis in a 30-year-old woman who was on systemic steroid therapy for active systemic lupus erythematosus. The patient was referred to our department with purple papules and ulcerated nodules on the dorsum of the hands, wrists and ankles. Skin biopsy showed granulomatous reaction with fungal elements that were subsequently identified as Alternaria species. Individual lesions were successfully treated with oral itracanozole 200mg daily for six weeks. Besides the patient's own disease, the use of systemic steroid use might be a possible predisposing factor for the development of cutaneous alternariosis.

Clinical features and natural course of Behçet's disease in 661 cases: a multicentre study.

BACKGROUND: Behçet's disease (BD) is a systemic inflammatory disease with unpredictable exacerbations and remissions. The natural course of BD is not fully known. OBJECTIVES: We aimed retrospectively to determine the occurrence of the symptoms in chronological order. We also evaluated the influence of the treatment and follow-up on the clinical severity and tried to identify the factors determining severe organ involvement. METHODS: Six hundred and sixty-one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient. RESULTS: Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85.3%), papulopustular lesions (55.4%), erythema nodosum (44.2%), skin pathergy reaction (37.8%), and articular (33.4%) and ocular involvement (29.2%). Oral ulcers were the most common onset manifestation (88.7%). The mean +/- SD duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4.3 +/- 5.7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0.001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy reaction were found to be significantly more frequent in males. CONCLUSIONS: Mucocutaneous lesions are the hallmarks of the disease, and especially oral ulcers precede other manifestations. The increase in clinical severity score is more pronounced in patients without regular treatment and follow-up. Male sex and a younger age at onset are associated with more severe disease.

Toll-like receptor 2 Arg753Gln gene polymorphism in Turkish patients with Behçet's disease.

Having considered the impact of the function of TLR2 in the recognition of several microorganisms that are thought to have an association with Behçet's disease (BD), we aimed to determine a possible association between the TLR2 Arg753Gln polymorphism and susceptibility to BD. We genotyped 83 patients with BD, 95 ethnically matched healthy controls, 12 patients with recurrent aphthous stomatitis (RAS) and 21 patients with rheumatoid arthritis (RA) by restriction fragment length polymorphism after PCR amplification of the genomic region encompassing the polymorphic site. Comparison of the TLR2 Arg753Gln A allele and A/G genotype frequencies did not show a significant difference between patients with BD and healthy controls (1.2% vs. 0.6%, and 2.1% vs. 1.1%, respectively). None of the patients from the RAS and RA groups had the A allele or A/G genotype. Our results indicate that the TLR2 Arg753Gln polymorphism does not play a role in the aetiopathogenesis of BD.

TNF-alpha gene 1031 T/C polymorphism in Turkish patients with Behçet's disease.

BACKGROUND: Genetic factors that predispose individuals to Behçet's disease (BD) are considered to play an important role in development of the disease. The tumour necrosis factor (TNF)-alpha gene, which is closely linked to the HLA-B51 gene, is involved in susceptibility for BD. Recently, a polymorphism at position -1031 within the TNF-alpha promoter region was demonstrated to be responsible for susceptibility to BD in a British population. However, the functional effects of this polymorphism have not yet been determined. OBJECTIVES: To investigate the possible relation of the TNF-alpha-1031 T/C polymorphism with susceptibility to BD in a Turkish population and to determine the functional importance of this polymorphism. METHODS: Ninety-nine unrelated patients (47 women, 52 men; mean +/- SD age, 34.10 +/- 10.53 years) with BD and 103 ethnically matched healthy controls (52 males, 51 females; mean +/- SD age, 40.25 +/- 14.15) were enrolled in the study. For genotyping, polymerase chain reaction - restriction fragment length polymorphism (PCR-RFLP) analysis was employed. The functional importance of TNF-alpha-1031 T/C polymorphism was determined with an enzyme-linked immunospot (ELISPOT) assay. For this purpose, mononuclear cells obtained from BD patients and controls were analysed for TNF-alpha and interferon (IFN)-gamma production. RESULTS: A significant difference was observed between BD patients and controls with respect to the allele frequency of TNF-alpha-1031C [P = 0.018, OR = 1.83, 95% confidence interval (CI) = 1.07-3.13]. When the allele frequencies were analysed according to the clinical features, the T allele in patients with positive skin pathergy test (SPT) was significantly increased when compared with those of patients without these findings (P = 0.004, OR = 2.75, 95% CI = 1.3-5.86). To demonstrate the frequency of TNF-alpha and IFN-gamma producing cells, mononuclear cells from four representative individuals of each genotype were used and the spontaneous and stimulated TNF-alpha and IFN-gamma values (spot numbers) were analysed. Compared with the control groups, a significant increase was observed in the number of cells producing TNF-alpha obtained from BD patients (P < 0.001). Moreover, the stimulation index for TNF-alpha [bacterial lipopolysaccharide (LPS) stimulated/unstimulated] was higher for the CC genotype (9 +/- 9.5) with respect to the other genotypes (TT; 1.3 +/- 0.3 and TC; 1.2 +/- 0.2). While the difference in the spontaneous IFN-gamma values between groups were not statistically significant, the stimulated IFN-gamma values were found to be significantly increased in the BD group when compared with the healthy control group (P = 0.004). CONCLUSIONS: Our results showed that, in the Turkish population the TNF-alpha-1031C allele is associated with susceptibility to BD. On the other hand, carrying the T allele may render patients more prone to developing a positive skin pathergy test. In addition, ELISPOT assays revealed that BD patients exhibited a significantly higher number of mononuclear cells producing TNF-alpha, and cells obtained from patients with a CC genotype had a stronger response to LPS stimulation. The strong IFN-gamma response upon LPS stimulation in BD patients supports the previous findings that BD is a Th1 driven disease. These findings suggest that the TNF-alpha-1031 polymorphism may have a functional effect and could explain the reason for high levels of TNF-alpha production observed in BD patients.