64 research outputs found

    Pyoderma gangrenosum.

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    Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that presents with rapidly developing, painful skin ulcers hallmarked by undermined borders and peripheral erythema. Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG is often associated with a variety of other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis. The cause of PG is not well understood, but PG is generally considered an autoinflammatory disorder. Studies have focused on the role of T cells, especially at the wound margin; these cells may support the destructive autoinflammatory response by the innate immune system. PG is difficult to diagnose as several differential diagnoses are possible; in addition to clinical examination, laboratory tests of biopsied wound tissue are required for an accurate diagnosis, and new validated diagnostic criteria will facilitate the process. Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) to reduce inflammation followed by the addition of more slowly acting immunosuppressive drugs with superior adverse event profiles, including biologics (in particular, anti-tumour necrosis factor (TNF) agents). Appropriate wound care is also essential. Future research should focus on PG-specific outcome measures and PG quality-of-life studies

    Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum : A Delphi Consensus of International Experts

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    IMPORTANCE Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a "diagnosis of exclusion," a definition not compatible with clinical decision making or inclusion for clinical trials. OBJECTIVE To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. EVIDENCE REVIEW Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation. FINDINGS Delphi exercise yielded 1 major criterion biopsy of ulcer edge demonstrating neutrophilic infiltrate and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or "wrinkled paper" scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively. CONCLUSIONS AND RELEVANCE. This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials

    Thermodynamics and Thermoelectricity

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    Thermoelectric (TE) effects result from the interference of electrical current and heat flow in various materials. This chapter presents a retrospective view on some of the milestones in the development of thermoelectricity. It reviews the thermodynamic theory of galvano- and thermomagnetic effects. As for TE materials, the galvano- and thermomagnetic effects can be found to be more pronounced in semiconductor materials. The chapter considers a basic thermodynamic system and thermodynamics of the ideal Fermi gas. Classical thermodynamics, which is useful for describing equilibrium states, provides very incomplete information on the actual physical phenomena, which are characterized by irreversibility and nonequilibrium states. Since the TE process implies the coupling of the heat flux and electric current, these two fluxes should be driven optimally. They derived two key parameters of the compatibility approach, the relative current and the TE potential

    Medulloblastoma, Primitive Neuroectodermal Tumors, and Pineal Tumors

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    The DNA Damage Response, DNA Repair, and AML

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    Prognosis of Cutaneous Lupus Erythematosus

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