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Background: Generalized pustular psoriasis (GPP) is a rare, inflammatory skin disease characterized by widespread eruption of sterile pustules with or without systemic symptoms. Objectives: This study aimed to describe the demographics of patients with GPP in Central and Eastern Europe (CEE), present the clinical characteristics of individual GPP flares and explore the current treatment landscape. Methods: Patient demographics were collected at the times of last observation and previous treatment. Characteristics of a patient's last (most recent) and most severe (from all documented episodes) flare were provided at clinician's discretion. Results: Fifty‐eight patients were recruited from 12 centres in nine CEE countries; median (range) age was 61 (16–92) years and 60.3% (35 out of 58) were female. The most common comorbidities were hypertension (43.1% [25 out of 58]) and hyperlipidaemia (32.8% [19 out of 58]). Thirty‐four patients (58.6%) presented with concomitant plaque psoriasis before or during the course of GPP. Data from two separate flares were recorded in 26 individuals; in 32 patients, the most recent flare was reported as the most severe. Over 90% of patients with a flare episode classified as most severe by clinicians were hospitalized, with >75% of these individuals having a Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) total score of 3 or 4. Systemic symptoms were more common in patients with a GPPGA score of 3 or 4 but were also manifest in individuals with a GPPGA score ≤2. A significant correlation was observed between a combined systemic disease score of clinical and laboratory features and both GPPGA total score (r = 0.385, p < 0.001) and GPPGA pustulation subscore (r = 0.305, p < 0.05). Conclusions: Considerable heterogeneity in the presentation of GPP flares was observed, both between patients and within‐patient. All GPP flares were associated with a significant clinical burden, highlighting the unmet need for accurate and early diagnosis. [ABSTRACT FROM AUTHOR]

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Background and Objectives : Methotrexate is widely prescribed for the treatment of moderate-to-severe psoriasis. As drug survival encompasses efficacy, safety, and treatment satisfaction, such studies provide insights into successful drug treatments in the real-life scenario. The objective was to define methotrexate drug survival and reasons for discontinuation, along with factors associated with drug survival, in a cohort of adult patients with moderate-to-severe plaque psoriasis. Materials and Methods : Data on methotrexate treatment were extracted from our institutional registry. Drug survival was estimated by Kaplan-Meier analysis, and predictors of drug survival were analyzed by Cox proportional hazards regression. Results : We included 133 patients treated with methotrexate. Due to significant effects of the year of treatment initiation, drug survival analysis was performed for 117 patients who started methotrexate in 2010 or later. Median methotrexate drug survival was 11.0 months. Overall, 89% of patients discontinued treatment, with over half of these (51%) due to lack of efficacy. Significantly longer drug survival was seen for patients who discontinued treatment due to lack of efficacy versus drug safety ( p = 0.049); when stratified by sex, this remained significant only for women ( p = 0.002). The patient ABCC2 rs717620 genotype was significantly associated with drug survival in both univariate log-rank and multivariate Cox regression analyses, with variant T allele associated with longer drug survival (hazard ratio, 0.606; 95% confidence interval, 0.380-0.967; p = 0.036). Conclusions : We have identified the novel association of patient ABCC2 rs717620 genotype with methotrexate drug survival. This pharmacogenetic marker might thus help in the management of psoriasis patients in daily practice.

Methotrexate is used as first-line treatment of moderate to severe psoriasis. Despite the marked variability in treatment outcomes, no pharmacogenetic markers are currently used for personalised management of therapy. In this retrospective study, we investigated the effects of genetic predisposition on efficacy and toxicity of low-dose methotrexate in a cohort of 137 patients with moderate to severe plaque psoriasis. We genotyped 16 polymorphisms in genes for enzymes involved in the folate-methionine pathway and in methotrexate transport, and analysed their association with treatment efficacy and toxicity using classification and regression tree analysis and logistic regression. The most pronounced effect observed in this study was for GNMT rs10948059, which was identified as a risk factor for inadequate efficacy leading to treatment discontinuation. Patients carrying at least one variant allele had ~7-fold increased risk of treatment failure compared to patients with the wild-type genotype, as shown by the classification and regression tree analysis and logistic regression (odds ratio [OR], 6.94; p = 0.0004). Another risk factor associated with insufficient treatment responses was DNMT3b rs2424913, where patients carrying at least one variant allele had a 4-fold increased risk of treatment failure compared to patients with the wild-type genotype (OR, 4.10; p = 0.005). Using classification and regression tree analysis, we show that DNMT3b rs2424913 has a more pronounced role in patients with the variant GNMT genotype, and hence we suggest an interaction between these two genes. Further, we show that patients with the BHMT rs3733890 variant allele had increased risk of hepatotoxicity (OR, 3.17; p = 0.022), which is the most prominent reason for methotrexate discontinuation. We also show that variants in the genes for methotrexate transporters OATP1B1 (rs2306283/rs4149056 SLCO1B1 haplotypes) and ABCC2 (rs717620) are associated with increased risk of treatment failure. The associations identified have not been reported previously. These data suggest that polymorphisms in genes for enzymes of the methionine cycle (which affect cell methylation potential) might have significant roles in treatment responses to methotrexate of patients with psoriasis. Further studies are warranted to validate the potential of the pharmacogenetic markers identified., (Copyright © 2021 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Background: Drug survival is an important measure of successful treatment of patients with chronic diseases such as psoriasis. Therefore, the objective was to calculate drug survival and examine safety profile of biologics and immunomodulators (adalimumab, apremilast, etanercept, ixekizumab, infliximab, secukinumab, and ustekinumab) from the Slovenian National Registry of patients with moderate-to-severe psoriasis., Methods: Data about the patients with moderate-to-severe plaque psoriasis treated with biologics were collected from 2005 until July 2018. Kaplan-Meier survival curves and Cox regression were used to calculate drug survival, where ustekinumab was selected as a reference., Results: Overall, 1,606 patients were analyzed within 2,241 treatment episodes; adalimumab N = 831, apremilast N = 94, etanercept N = 101, ixekizumab N = 98, infliximab N = 164, secukinumab N = 340, and ustekinumab N = 613, respectively. Loss of efficacy was the most frequent reason for treatment discontinuation (contributing to 66.1% of all discontinuations). Ustekinumab was associated with the highest drug survival, meanwhile apremilast was the drug with the lowest survival rate compared to all others. Both IL-17 inhibitors, secukinumab and ixekizumab, showed similar survival rate., Conclusions: Ustekinumab was associated with the highest drug survival and most favorable safety profile compared to other biologics. Drug survival rates can be associated with the class effect of biological targets. Highest survival rate was observed for IL-12/23 inhibitor, followed by IL-17 and TNF-α inhibitors, and last by an immunomodulator such as apremilast. Adverse events occurred most frequently with TNF-α inhibitors., (© 2019 The International Society of Dermatology.)

Syringotropic mycosis fungoides (STMF) is an extremely rare form of cutaneous T‑cell lymphoma with 51 published cases so far. Clinically STMF is manifested similarly to folliculotropic mycosis fungoides (MF), whereby the course of STMF is much milder. Histopathologically, it shows a prominent tropism of the T‑cell lymphocytic infiltrate for the eccrine epithelium. We report the case of a 65-year-old woman with multiple small papules on the feet, shinbones and back.

Chronic peripheral edema is a very common yet underestimated problem. It is of utmost importance to investigate various possible causes and understand the pathophysiological mechanisms of chronic peripheral edema in order to determine the optimal treatment. This case report presents the case of a polymorbid patient with a deformity-causing type of rheumatoid arthritis and a 1-year history of progressive symmetrical peripheral edema. Based on an extensive diagnostic investigation, the patient was diagnosed with a combined form of chronic peripheral edema comprised of true lymphedema, which was confirmed by lymphoscintigraphy, and filtration edema due to muscular inactivity. Edema as a side effect of tocilizumab was also suspected.

Rare clinical variants of bullous pemphigoid (BP) include vesicular BP, dyshidrosiform BP, pemphigoid nodularis, seborrheic BP, pemphigoid vegetans, localized BP, erythrodermic BP, and juvenile BP. To our knowledge, this is the first report of an unusual case of purpuric BP. We present a case of 85-year-old white man who presented with a 2-week history of blisters and pruritic urticarial lesions all over his body. The diagnosis of purpuric BP was made on the basis of history, clinical presentation, histopathology report, direct and indirect immunofluorescence studies the diagnosis of purpuric BP was made. The reason for the development of palmoplantar purpuric lesions concomitant to ordinary patches and plaques of BP is unknown.

Zinc deficiency can be an autosomal recessive inherited or acquired disorder and is classically characterized by acral and periorificial dermatitis, alopecia, and diarrhea. We report a case of acquired zinc deficiency in a premature 6-week-old boy with typical skin manifestation of zinc deficiency and decreased plasma zinc level. After starting zinc replacement therapy, the skin lesions completely disappeared in the first few weeks.

Post-steroid panniculitis is an extremely rare phenomenon caused by inappropriate interruption of long-term systemic corticosteroid therapy. It usually occurs in children and is characterized by development of multiple subcutaneous nodules on the cheeks, arms, and trunk. Histologically it is a lobular type of panniculitis with characteristic needle-shaped clefts within adipocytes and numerous foreign-body giant cells. We present a case of post-steroid panniculitis occurring in a 50-year-old female after long-term administration of oral corticosteroids for Sjögren's syndrome accompanied by leukocytoclastic vasculitis and chronic polyarthritis.

Cutaneous leishmaniasis is the most common form of leishmaniasis caused by flagellate protozoa of the genus Leishmania transmitted by sand fly bites. Old World leishmaniasis is endemic in the Mediterranean Sea and the neighbouring countries. We present a case of a 10-year-old boy with a cutaneous leishmaniasis in form of papules on the nose, right and left lower leg. Histopathological examination showed diffuse dermal infiltrate predominantly of macrophages with admixture of few lymphocytes, eosinophils and plasma cells. In most of macrophages amastigotes were seen. Because of higher rate of travel and work abroad increased number of sporadic cases of cutaneous leishmaniasis in non-endemic areas should be taken into account.