Arch Dermatol
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Stiff skin syndrome is a sclerodermalike disorder that presents in infancy or early childhood with rock-hard skin, limited joint mobility, and mild hypertrichosis in the absence of visceral or muscle involvement, immunologic abnormalities, or vascular hyperreactivity. ⋯ Stiff skin syndrome is characterized by an early, insidious onset of stony-hard skin, often with associated contracturelike joint restriction, hypertrichosis, and postural and thoracic wall abnormalities. Supportive histopathologic findings consisting of either fascial sclerosis or increased fibroblast cellularity with sclerotic collagen bundles in the deep reticular dermis and/or subcutaneous septa may help to confirm this diagnosis.
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We conducted a literature review to investigate the recent advances in genetics, molecular biology, clinical manifestations, and therapy of 7 inherited diseases that are characterized by seemingly unprovoked inflammation. These autoinflammatory diseases include familial Mediterranean fever; tumor necrosis factor receptor-associated periodic syndrome; hyperimmunoglobulinemia D with periodic fever syndrome; pyogenic arthritis, pyoderma gangrenosum, and acne syndrome; and the 3 cryopyrinopathies: neonatal-onset multisystem inflammatory disease/chronic infantile neurologic cutaneous and arthropathy syndrome, familial cold autoinflammatory syndrome, and Muckle-Wells syndrome. Recent identification of the susceptibility genes for autoinflammatory diseases has broadened the clinical spectrum as well as the molecular basis of these diseases. ⋯ Recent advances in genetics and molecular biology have advanced our understanding of the pathogenesis of autoinflammatory diseases. Understanding autoinflammatory diseases will further our knowledge of cutaneous as well as systemic inflammation. Anakinra, a recombinant human interleukin 1 receptor antagonist, is a promising new biologic agent for the treatment of cryopyrinopathies as well other autoinflammatory diseases, such as tumor necrosis factor receptor-associated periodic syndrome and hyperimmunoglobulinemia D with periodic fever syndrome.
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To critically review the literature on the efficacy of modern dressings in healing chronic and acute wounds by secondary intention. ⋯ Our systematic review provided only weak levels of evidence on the clinical efficacy of modern dressings compared with saline or paraffin gauze in terms of healing, with the exception of hydrocolloids. There was no evidence that any of the modern dressings was better than another, or better than saline or paraffin gauze, in terms of general performance criteria. More wound care research providing level A evidence is needed.
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To make preliminary recommendations for defining a flare of atopic dermatitis (AD) in clinical research based on a systematic review of the literature and experience in running clinical trials. ⋯ Defining an AD flare is a complex process, and this review has highlighted the need for standardization in defining measures of long-term disease control. We propose that a flare of AD be simply defined as an episode requiring escalation of treatment or seeking additional medical advice. Consideration should also be given to totally controlled weeks and well-controlled weeks to assess overall disease activity in patients with AD. Together, these definitions are intuitive, simple to use, and easy to understand. Future work is required to test the applicability of these recommendations in a variety of research settings.
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To make preliminary recommendations for defining a flare of atopic dermatitis (AD) in clinical research based on a systematic review of the literature and experience in running clinical trials. ⋯ Defining an AD flare is a complex process, and this review has highlighted the need for standardization in defining measures of long-term disease control. We propose that a flare of AD be simply defined as an episode requiring escalation of treatment or seeking additional medical advice. Consideration should also be given to totally controlled weeks and well-controlled weeks to assess overall disease activity in patients with AD. Together, these definitions are intuitive, simple to use, and easy to understand. Future work is required to test the applicability of these recommendations in a variety of research settings.