Aleksandra Batycka-Baran

Dowling-Degos Disease: Case Report and Review of the Literature

Dowling-Degos disease (DDD) is an unusual pigmentary disorder usually caused by mutations in keratin 5. A 44-year-old woman in good general health presented due to the recent appearance of numerous pigmented macules on her axillary and anogenital skin. A biopsy showed lacy, finger-like epidermal extensions into the dermis which were heavily pigmented and associated with tiny cysts or dilated follicles. We view DDD as part of a spectrum of disorders which are morphologically related but vary in location and time of expression. In addition, both the clinical and histological differential diagnostic considerations are extensive.

Reduced Number of Circulating Endothelial Progenitor Cells (CD133+/KDR+) in Patients with Plaque Psoriasis

Background: Psoriasis is associated with an increased cardiovascular risk. Circulating endothelial progenitor cells (CEPCs) play a significant role in the maintenance of vascular homeostasis. Objective: The aim of this study was to evaluate the number of CEPCs in patients with psoriasis compared to controls and assess possible correlations between the number of these cells and the plasma levels of vascular endothelial growth factor (VEGF), soluble vascular endothelial growth factor receptor-1 (sVEGFR-1) and clinical features of psoriasis. Methods: The number of CEPCs, identified as CD133+/KDR+ cells, was determined with flow cytometry in peripheral blood of psoriatic patients (n = 63) and controls (n = 31). The plasma levels of VEGF and sVEGFR-1 were measured with enzyme-linked immunosorbent assay. Results: The number of CEPCs was significantly reduced in psoriatic patients compared with controls (p = 0.000026) and inversely correlated with disease severity (R = –0.283; p = 0.0248). Conclusion: A reduced number of CEPCs may contribute to endothelial dysfunction in patients with psoriasis.

Increased number of circulating endothelial cells (CECs) in patients with psoriasis ‐ preliminary report

Background  Numerous studies have demonstrated increased cardiovascular risk in psoriasis. Circulating endothelial cells (CECs) have been proposed as a new marker of endothelial dysfunction that plays an important role in pathogenesis of atherosclerosis.

Folate supplementation reduces the side effects of methotrexate therapy for psoriasis.

Introduction: Methotrexate (MTX) is an effective treatment option for patients with moderate-to-severe psoriasis. As psoriasis is an incurable disease, the goal of the MTX treatment is to suppress psoriasis, achieving long-term remissions with minimal treatment-related adverse events (AEs). Areas covered: Supplementation with folate – either folic acid (FA) or folinic acid – may reduce the side effects of MTX therapy. There are no consistent, evidence-based guidelines for folate supplementation in this clinical setting. We present data concerning the impact of folic supplementation on the safety and efficacy of MTX therapy for psoriasis. An extensive search (1960 – March 2014) identified few studies addressing with just one randomized controlled trial (RCT) and some case series. This meager yield underlines the need for further studies, especially RCTs, in this group of patients. Expert opinion: FA may be effective in diminishing the severity of AEs in patients with psoriasis treated with MTX and should be recommended. Supplementation with too high doses of FA may influence the efficacy of treatment.

Th17 micro-milieu regulates NLRP1-dependent caspase-5 activity in skin autoinflammation

IL-1β is a potent player in cutaneous inflammation and central for the development of a Th17 micro-milieu in autoinflammatory diseases including psoriasis. Its production is controlled at the transcriptional level and by subsequent posttranslational processing via inflammatory caspases. In this study, we detected inflammatory caspase-5 active in epidermal keratinocytes and in psoriatic skin lesions. Further, interferon-γ and interleukin-17A synergistically induced caspase-5 expression in cultured keratinocytes, which was dependent on the antimicrobial peptide psoriasin (S100A7). However, diseases-relevant triggers for caspase-5 activity and IL-1β production remain unknown. Recently, extranuclear DNA has been identified as danger-signals abundant in the psoriatic epidermis. Here, we could demonstrate that cytosolic double-stranded (ds) DNA transfected into keratinocytes triggered the activation of caspase-5 and the release of IL-1β. Further, interleukin-17A promoted caspase-5 function via facilitation of the NLRP1-inflammasome. Anti-inflammatory vitamin D interfered with the IL-1β release and suppressed caspase-5 in keratinocytes and in psoriatic skin lesions. Our data link the disease-intrinsic danger signals psoriasin (S100A7) and dsDNA for NLPR1-dependent caspase-5 activity in psoriasis providing potential therapeutic targets in Th17-mediated skin autoinflammation.

The effect of phototherapy on systemic inflammatory process in patients with plaque psoriasis

Psoriasis is a common, chronic immune-mediated inflammatory disease. The inflammatory process in psoriasis has systemic effects and may influence the development of psoriatic comorbidities. The systemic action of phototherapy in patients with psoriasis has been so far poorly elucidated. We aimed to investigate the expression of genes encoding selected psoriasis-related cytokines in peripheral blood mononuclear cells (PBMCs) isolated from patients with psoriasis before and after treatment with phototherapy. 17 patients with mild to moderate plaque psoriasis were treated with narrow band-UVB (NB-UVB), 8 patients with moderate to severe plaque psoriasis with bath-psoralen-ultraviolet A therapy (PUVA). PBMCs were isolated by Ficoll gradient density centrifugation. Expression of genes encoding TNF-α, IL-17A, IL-6, IL-1 β, INF-γ, and IL-10 in PBMCs of patients with psoriasis before and after phototherapy was analyzed with quantitative RT-PCR. Treatment with NB-UVB therapy led to a significant decrease in IL-17A, TNF-α, and IL-6 mRNA levels in PBMCs (p=0.003; p=0.042; p=0.019, respectively). Following treatment with bath-PUVA therapy, we observed a significant decrease in TNF-α and IL-6 mRNA levels in PBMCs (p=0.031, p=0.035, respectively). Treatment with phototherapy in patients with psoriasis may affect systemic inflammation by downregulation of the expression of genes encoding proinflammatory cytokines in PBMCs, implicated in the development of psoriasis and psoriatic comorbidities.

Decreased Number of Circulating Endothelial Progenitor Cells in Hidradenitis Suppurativa Patients

Background: Hidradenitis suppurativa (HS) is nowadays regarded as a systemic disease associated with metabolic syndrome. Some recent studies have also demonstrated an increased cardiovascular risk in patients with HS. Circulating endothelial progenitor cells (EPCs) play an integral role in the regulation and protection of the endothelium and in the maintenance of vascular homeostasis. Objective: This study was undertaken to determine the possible alterations in the number of EPCs in patients with HS compared to controls. Methods: The number of EPCs, identified as CD133+/KDR+ cells, was determined with flow cytometry in the peripheral blood of 25 HS patients and 31 controls. Results: The number of EPCs was significantly reduced in HS sufferers compared with controls (p < 0.0001). The mean number of EPCs was assessed as 191.3 ± 118.5/ml and 672.4 ± 343.0/ml for patients and controls, respectively. Conclusion: A decreased number of EPCs among HS sufferers may contribute to endothelial malfunction resulting in increased cardiovascular risk in this group of patients.

Shiitake Dermatitis - Now Also in Poland

Shiitake dermatitis is a striking skin reaction that may appear after consumption of raw or undercooked Shiitake mushrooms (Lentinus edodes) in susceptible individuals. The disease is also termed flagellate mushroom dermatitis because the skin lesions resemble wounds caused by whip (flagellum in Latin) (1, 2). It was first described in Japan by Nakamura (3) in 1977 and to date only few cases were reported in Europe (2, 4, 5).

Effective treatment of leukemia cutis with combination of rituximab, cladribine, and cyclophosphamide in patient with B-cell chronic lymphocytic leukemia

*Department of Dermatology, Venereology a nd Allergology Wroclaw Medical University, Poland ^Department of Hematology , Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Poland Corresponding author: Wojciech Baran - Department of Dermatology, Venereology and Allergolo gy, Wroclaw Medical University, ul.Chalubinskiego 1, 50 -368,Wroclaw, Poland, e-mail : wbaran@mp.pl tel. 0048713270941 ; fax. 0048713270942

Decreased Number of Circulating Endothelial Progenitor Cells (CD133+/KDR+) in Patients with Psoriatic Arthritis.

Cardiovascular diseases are a major cause of mortality in patients with psoriatic arthritis (PsA), but the precise mechanism of increased cardiovascular risk is unknown. Endothelial dysfunction plays a crucial role in the development of atherosclerosis. Circulating endothelial progenitor cells (CEPCs) contribute to endothelial regeneration and their level may be affected by chronic inflammation. The aim of this study was to evaluate the number of CEPCs in patients with PsA (n = 24) compared with controls (n = 26). CEPCs were identified as CD133+/ KDR+ cells in peripheral blood, using flow cytometry. A significantly decreased number of CEPCs was observed in patients with PsA (p < 0.0001). The number of these cells was significantly, inversely correlated with the severity of skin and joint involvement (Psoriasis Area and Severity Index (PASI), DAS28) and the level of C-reactive protein. We hypothesize that the reduced number of CEPCs may indicate and contribute to the increased cardiovascular risk in patients with PsA.