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.

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.

Evaluation of eye health in children with type 1 diabetes mellitus and celiac disease.

Background: Pediatric celiac disease (CeD) and type 1 diabetes mellitus (T1DM) have well established effects on eye health but comorbid effect is not known. Aim: To evaluate the eye health of children with T1DM and CeD to predict microvascular retinal pathologies by diagnosis of probable intraocular pressure increase which is an important glaucoma trigger. Patients and Methods: In this case-controlled study, 28 eyes of 14 children both T1DM and CeD, with a mean age of 12.6 ± 3.9 years, and 28 eyes of gender-matched 14 healthy children as a control group were included. In both groups, detailed ocular examinations and measurement of intraocular pressure (IOP), ocular pulse amplitude (OPA), thicknesses of ganglion cell layer (GCL), inner plexiform layer (IPL), retinal nerve fiber layer (RNFL), and choroid thicknesses (CT) were done. All the patients with T1DM and CeD were newly diagnosed. The evaluations of IOP and OPA were made using a Pascal dynamic tonometer and thicknesses measured by optical coherence tomography. Results: The IOP and OPA values of the patient group were found to be statistically significantly higher than those of the control group (17.1 and 1.86 vs 14.78 and 1.57 mmHg, P <.0001, P <.001, respectively). IOP values of all patients were higher than IOP cut off levels for diagnosis of hypertension. CT was significantly thinner in the patient group than in the control group (385.4 µm vs 331.71 µm, respectively, P < 0.03). No significant difference was found between the groups in respect of GCL, IPL, and RNFL values. Conclusion: The higher IOP and OPA values of the children with T1DM and CeD were considered to be the result of the microvascular pathologies in T1DM and increased inflammation associated with CeD. High IOP and OPA values can lead to damage in the eye as intraocular blood flow and choroidal perfusion are affected. In order to prevent these eye problems, measurement of IOP and OPA should be done in children with diagnosis of T1DM and CeD and also follow up studies needed.

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.

Antibiotic resistance in acne: changes, consequences and concerns.

Antibiotic resistance in acne was first observed in the 1970s and has been a major concern in dermatology since the 1980s. The resistance rates and types of antimicrobials have subsequently shown great variations in regions and countries. Illustrative of this is the resistance to topical erythromycin and clindamycin which continues to be a problem worldwide, while resistance to systemic treatment with tetracyclines has remained low during the past decade. The resistance for the newer macrolides like azithromycin and clarithromycin has been increasing. The results of antibiotic resistance may include treatment failure of acne, disturbance of skin microbiota, induction of opportunistic pathogens locally and systemically, and dissemination of resistant strains to both healthcare personnel and the general population. The ensuing complications, such as aggravated opportunistic infections caused by Propionibacterium acnes and the emergence of multiresistant superbugs, have not yet been confirmed.

Approach to skin malignancies in COVID-19 pandemic days.

COVID-19 pandemic emerged as a condition that changed our entire life and led us to develop new perspectives on diseases. Cancers are generally additional risk factors for COVID-19, and this has implications also for skin cancer patients. Those patients require special attention, due to situations such as an increased risk of infection as a result of visiting the hospital for treatment. It is imperative that the diagnosis and treatment of patients who have a cancer that progresses rapidly, such as malignant melanoma, high-risk squamous cell carcinoma and a high risk of metastasis, are delayed. Due to the relatively long-lasting nature of basal cell carcinoma and, as almost no metastasis would be expected, its treatment may be postponed, except for those that occur in areas such as the eyes and mouth. Diagnosis and treatment of skin cancers is a process that requires many procedures. Throughout this process, physicians should take appropriate precautions that are sufficient to protect both the patient and themselves in the best way without leading to any delay in the procedures.

ICHTHYOSIS LINEARIS CIRCUMFLEXA: NOT ALWAYS A PART OF NETHERTON SYNDROME.

Ichthyosis Linearis Circumfiexa (ILC) is a rarely seen autosomal recessive keratinization disorder and is characterized by erythematous, polycystic, plaques with 'double-edged' scales. Its histological features resemble psoriasis. A triad of ichthyosis (usually Ichthyosis Linearis Circumfiexa), trichorrhexis invaginata (a distinctive hair shaft disorder) and atopy is named as Netherton syndrome. Herein, we report a 12 year-old girl presenting with ILC not accompanied with typical atopy findings and hair shaft disorder.

Decreased serum paraoxonase and arylesterase activities in patients with rosacea.

BACKGROUND: Recent evidence suggests that oxidative stress may be an important phenomenon in the pathophysiology of rosacea. Paraoxonase-1 (PON1) is an antioxidant enzyme with three activities: paraoxonase, arylesterase and dyazoxonase. In this study, we evaluated serum paraoxonase and arylesterase activities, and serum lipid hydroperoxide (LOOH) levels in patients with rosacea in comparison to healthy controls. MATERIAL AND METHOD: The study included 39 rosacea patients and healthy controls, consisting of 40 age- and sex-matched healthy volunteers. Serum paraoxonase and arylesterase activities were measured using paraoxon and phenylacetate substrates. Serum LOOH levels were measured with the ferrous ion oxidation-xylenol orange assay. RESULTS: In rosacea group mean serum paraoxonase and arylesterase activities were 74.54 ± 38.30 U L(-1) and 141.29 ± 22.27 kU L(-1) respectively, which were significantly lower than controls (P = 0.010, 0.005; respectively). Mean serum LOOH level of rosacea group was 8.17 ± 1.91 µmol L(-1) which was significantly higher than controls (P = 0.009). There were no statistically significant differences between the clinical subtypes of the disease, menopause situation or ocular involvement with the respect to the serum paraoxonase and arylesterase activities and LOOH levels (all; P > 0.05). CONCLUSIONS: Serum PON1 enzyme activities have decreased significantly in rosacea. These findings support that decreased PON1 activity and increased oxidative stress may play a role in the pathogenesis of rosacea. Further studies are needed to elucidate the role of PON1 activity in the pathophysiology of rosacea.

Elevated insulin resistance in patients with recurrent aphthous stomatitis.

OBJECTIVES: The role of glucose metabolism disorders in periodontal diseases including recurrent aphthous stomatitis (RAS) is currently attracting attention. The aim of this study is to investigate insulin resistance (IR) in patients with RAS in otherwise healthy individuals. MATERIALS AND METHODS: The study enrolled 81 patients with RAS and 61 healthy control subjects. Blood glucose, insulin, C-peptide, hemoglobin A1c, total cholesterol (TC), high-density lipoprotein (HDL) cholesterol, and HOMA-IR were measured in each individual. RESULTS: Forty-two of the RAS group were in the active, and 39 of the RAS group were in the passive stage. The levels of c-peptide, insulin, and HOMA-IR were remarkably higher in the RAS group (p = 0.015; p < 0.0001; p < 0.0001, respectively) than the control group. The levels of HbA1c, glucose (p = 0,045), TC (p = 0,008), HDL cholesterol (p = 0,002), and HOMA-IR (p = 0.022) were significantly higher in patients with RAS in the active stage. CONCLUSION: The study revealed an elevated IR in patients with RAS that was not previously shown. IR was more prominent when the patients were in the active stage that elevated inflammatory cytokines may induce IR by interfering with insulin signaling. Further studies, investigating the interplay between RAS, inflammation, and IR are needed. CLINICAL RELEVANCE: Patients who admitted the hospital with RAS might be screened for prediabetes.

GAPO syndrome associated with pyoderma vegetans: an unreported co-existence.

GAPO syndrome is a rare autosomal recessive disease and an acronym composed of growth retardation, alopecia, pseudoanodontia, optic atrophy. Approximately 38 cases have been reported in literature until now. Pyoderma vegetans is a chronic inflammatory disease characterized with vesicopustular, exudative and vegetative lesions usually localized on face, scalp, axilla and genitalia. Pyoderma vegetans is attributed to a bacterial infection frequently occurring in individuals with an underlying immunosuppressive condition. A 30-year-old female patient was admitted to our clinic with complaint of a hemorrhagic, crusted, exudative vegetative two plaques on the scalp. On her physical examination, she had a prematurely aged face, predominant lower lips, total tooth loss, frontal bossing, enlarged anterior fontanelle, marked scalp veins, micrognatia, depressed nasal bridge, short stature, growth retardation. She was diagnosed as GAPO syndrome as the result of her physical examination. Result of the biopsy taken from scalp was evaluated as pyoderma vegetans. And the diagnosis of pyoderma vegetans was established based on the correlate of both clinical and histopathologic findings. Pyoderma vegetans coexisting with GAPO syndrome has not been reported previously. Thus we wished to report it.

A lamotrigine induced toxic epidermal necrolysis in a child.

Toxic epidermal necrolysis (TEN) is rare, life-threatening skin disorder that usually is caused by an adverse drug reaction. The exact pathogenesis of TEN is still unknown. Many treatments including prednisolone, cyclosporine and intravenous immunoglobulin (IVIG) can be used to halt the disease process. We present a 12-year-old girl with epilepsy who developed TEN after about 14 days of lamotrigine treatment. Lamotrigine was immediately discontinued. After receiving systemic corticosteroid treatment, the patient had a complete recovery. Antiepileptic-induced TEN can be mortal in some cases. Thus, we would like to point out the importance of early diagnosis and treatment.

Immunoregulatory effects of isotretinoin in patients with acne.

BACKGROUND: In vitro studies have shown that retinoids influence T-cell differentiation. OBJECTIVES: To study the effect of isotretinoin on T-cell differentiation markers in patients with acne. METHODS: A total of 37 patients with acne vulgaris (25 female, 12 male, age 19.6 ± 3.7 years) and 30 age- and sex-matched healthy controls (19 female, 11 male, age 20.5 ± 4.4 years) were included in the study. Screening for biochemical parameters in serum samples were done just before initiation (pretreatment) and after 3 months of isotretinoin treatment (post-treatment) in the acne group. RESULTS: Baseline levels of tumour necrosis factor (TNF)-α (P<0.0001), interleukin (IL)-4 (P<0.0001), IL-17 (P<0.0001) and interferon (IFN)-γ (P=0.002) were significantly higher in the acne group compared with the control group. TNF-α (P<0.0001), IL-4 (P<0.0001), IL-17 (P<0.0001) and IFN-γ (P<0.0001) levels decreased after isotretinoin treatment. TNF-α and IL-4 values after isotretinoin treatment were similar to those of the control group. However, levels of IL-17 (P<0.0001) after isotretinoin treatment were higher than those of the control group, despite a significant decline after treatment. Levels of IFN-γ (P<0.0001) after isotretinoin treatment were lower than those of the control group. CONCLUSIONS: This study shows that isotretinoin treatment significantly decreases TNF, IL-4, IL-17 and IFN-γ levels in patients with acne. We failed to show that isotretinoin redirects naive T helper (Th) differentiation preferentially towards the Th2 cell lineage.

Serum holotranscobalamine, vitamin B12, folic acid and homocysteine levels in patients with vitiligo.

Few studies have investigated the role of vitamin B12 metabolism in vitiligo. We tested the hypothesis that vitamin B12 and folate metabolism might have an influence on the pathogenesis of vitiligo. Full blood count and levels of folic acid, vitamin B12, homocysteine and holotranscobalamine were examined for 69 patients with vitiligo and 52 controls. The vitiligo group had higher levels of homocysteine (P < 0.01) and haemoglobin (P < 0.01) levels, and lower levels of vitamin B12 (P < 0.01) and holotranscobalamine (P < 0.0001) than the control group. Folic acid levels were similar for both groups. In a risk analysis, hyperhomocysteinaemia (≥ 15 µmol/L, P < 0.01) and vitamin B12 deficiency (< 200 pg/mL, P < 0.01) were significant risk factors for vitiligo. Patients with holotranscobalamine levels in the lowest quartile had an increased risk for co-occurrence of vitiligo (P < 0.005). Vitamin B12 deficiency and hyperhomocysteinaemia may share a common genetic background with vitiligo.

Thyroid autoimmunity associated with recurrent aphthous stomatitis.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is an autoimmune disorder characterized by the periodic appearance of aphthous lesions on the oral mucosa. TH1 cytokines plays a key role in the aetiopathogenesis. Autoimmune thyroid disease (ATD) is the most common autoimmune disease and is frequently accompanied by various other autoimmune diseases. OBJECTIVE: To investigate the frequency of ATD which has not been studied in the patients with RAS. METHODS: Ninety patients and 30 healthy volunteers were included into the study. The serum samples were assayed for thyroid stimulant hormone (TSH), free and total triiodothyronine (fT3, TT3), free and total thyroxine (fT4, TT4), thyroglobuline, anti-thyroid peroxidase antibody (anti-TPO) and anti-thyroglobuline antibody (anti-TG) levels. Thyroid ultrasonography was performed as well. RESULTS: In RAS patients, the fT3, TT3 levels were higher; whereas the fT4 levels were lower that the control group (P < 0.05). The anti-thyroid antibody was positive in 31.11% of the patients with RAS, and in only 10% of the individuals in the control group (P < 0.05). The mean anti-TG level was also higher in the RAS group. Ultrasonography revealed nodules in 28.8% of the patients with RAS and in 16.7% of the individuals in the control group (P < 0.05). The sT4 levels were lower and the TSH, anti-TPO and anti-TG levels were significantly higher in the RAS patients with thyroid nodules than the RAS patients without nodules (P < 0.05). DISCUSSION: These results may be related to either the advance age of the patients or the increased duration of the autoimmune activation which may affect the thyroid. CONCLUSIONS: The frequency of thyroid autoimmune-related problems was higher in patients with RAS. It would be worthy of searching autoimmune thyroid disorders in patients with RAS.

Incontinentia pigmenti with ocular involvement: two cases.

Incontinentia pigmenti (IP) is a rare, X-linked dominant disorder that presents at or soon after birth and presents with cutaneous signs and symptoms. Besides its typical skin signs presenting in all patients, central nervous system (CNS), ophthalmologic and dental involvement are encountered as well. In this report, we present two patients at the verrucous stage of IP and review the literature concerning the diagnosis and course of IP.

Isotretinoin does not induce insulin resistance in patients with acne.

BACKGROUND: Isotretinoin treatment causes hypertriglyceridaemia. Insulin resistance is also associated with hypertriglyceridaemia. It is not known if isotretinoin is related to insulin resistance. AIM: To test this hypothesis, we measured insulin resistance in 48 patients with acne vulgaris (AV) before and after 3 months of isotretinoin treatment. METHODS: In total, 48 patients with AV who attended the dermatology outpatient clinic at Kecioren Research and Training Hospital were included. Screening for biochemical parameters was performed just before the start of treatment (pretreatment) and after 4 months of isotretinoin therapy (post-treatment). The parameters measured were insulin, C peptide, fasting blood glucose, aspartate and alanine aminotransferases (AST, ALT), total cholesterol (TC), triglyceride, high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein (LDL-C) and very low-density lipoprotein cholesterol. Insulin resistance was measured using the Homeostasis Model Assessment of Insulin Resistance (HOMA-IR) method. RESULTS: Compared with initial values, AST, ALT, TC, LDL-C and triglyceride levels were significantly increased (P < 0.01, < 0.05, < 0.01, < 0.05 and < 0.01, respectively), but there was no significant change in fasting blood glucose, insulin, C-peptide levels or HOMA-IR. CONCLUSIONS: Three months of isotretinoin treatment did not change insulin sensitivity in patients with AV. Further studies with insulin resistance models may even reveal an improvement in insulin resistance, as experimental animal studies have previously shown.

Expression of GSTM4 and GSTT1 in patients with Tinea versicolor, Tinea inguinalis and Tinea pedis infections: a preliminary study.

BACKGROUND: Several skin diseases are believed to be associated with oxidative stress. Defence against reactive oxygen species in the skin involves a variety of antioxidant enzymes, including glutathione-S-transferases (GSTs) catalysing the reaction between reduced glutathione, and a variety of exogenously and endogenously derived electrophilic compounds. The mammalian soluble GSTs are divided into five main classes: alpha (A), mu (M), pi (P), theta (T) and zeta (Z). AIM: To investigate the expression of GSTM4 and GSTT1 in lesional and nonlesional skin of patients with dermatophytoses and Tinea versicolor infection. Methods. Expression of GSTM4 and GSTT1 was assessed by immunohistochemistry for dermatophytoses in 15 patients with T. versicolor, 15 patients with Tinea pedis and 8 patients with Tinea inguinalis, and compared with healthy controls (n = 9). After written consent was signed by each participant, punch biopsies were excised from the centre of the lesional skin sites in patients and from the normal skin sites in controls. The relationships between expression of GSTM4 and GSTT1 isoenzymes and fungal infections were also examined. RESULTS: When the normal and infected tissue of these cases were compared according to their staining intensity, GSTM4 expression was found to be stronger in control epithelium than in the epithelium of patients with T. pedis, T. inguinalis or T. versicolor (P < 0.05). By contrast, expression of GSTT1 was stronger in the epithelium of patients infected with any of the three dermatophytes than in control epithelium (P < 0.05). CONCLUSIONS: There is a significant relationship between presence of T. versicolor, T. inguinalis and T. pedis and expression of GSTM4 and GSTT1.