Acquired cutis laxa in childhood Sweet's syndrome.
ABSTRACT In Sweet's syndrome, the essential features are the characteristic morphology of the lesions, their histologic appearance, the dramatic response to corticosteroids and the absence of scarring. We report an 8-month-old infant in whom Sweet's syndrome was diagnosed and who developed acquired cutis laxa in the skin lesions.
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ABSTRACT: Elastic fibers are important components of the skin and are responsible for skin elasticity. Genetic defects are well-known in numerous hereditary elastic tissue disorders and skin biopsies are often the first step in the evaluation of those disorders. Verhoeff-Van Gieson elastic staining is a simple method that is used for visualizing elastic fibers. With the development of modern immunohistochemical methods, the value of routine histochemical staining is sometimes underestimated. Histochemical stains are less expensive, easy to perform and help to resolve numerous diagnostic quandaries in dermatopathology. This article focuses on the value of elastic tissue staining in dermatopathology, with a focus on primary elastic tissue disorders, alopecia, inflammatory skin disorders and neoplastic proliferations.Journal of Cutaneous Pathology 12/2012; · 1.56 Impact Factor
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ABSTRACT: Elastic fibres are insoluble components of the extracellular matrix of dynamic connective tissues such as skin, arteries, lungs and ligaments. They are laid down during development, and comprise a cross-linked elastin core within a template of fibrillin-based microfibrils. Their function is to endow tissues with the property of elastic recoil, and they also regulate the bioavailability of transforming growth factor β. Severe heritable elastic fibre diseases are caused by mutations in elastic fibre components; for example, mutations in elastin cause supravalvular aortic stenosis and autosomal dominant cutis laxa, mutations in fibrillin-1 cause Marfan syndrome and Weill-Marchesani syndrome, and mutations in fibulins-4 and -5 cause autosomal recessive cutis laxa. Acquired elastic fibre defects include dermal elastosis, whereas inflammatory damage to fibres contributes to pathologies such as pulmonary emphysema and vascular disease. This review outlines the latest understanding of the composition and assembly of elastic fibres, and describes elastic fibre diseases and current therapeutic approaches.Expert Reviews in Molecular Medicine 01/2013; 15:e8. · 5.91 Impact Factor
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ABSTRACT: Diagnosis of paraneoplastic skin syndromes associating neoplastic processes is assumed as the crucial aspect of dermatological practice. Knowledge of clinical findings of dermatoses suggesting coincidence of malignant proliferative processes facilitates diagnostic and therapeutic procedures. We would like to present a case of Sweet's syndrome, qualified for comparative paraneoplastic skin syndromes. Sweet's syndrome, acute, febrile neutrophilic dermatosis, was first described by Robert Douglas Sweet in 1964 as a disorder characterized by fever, skin lesions of erythematous-infiltrative character, leukocytosis with neutrophilia and dense infiltrations of dermis by mature neutrophils. Sweet's syndrome aetiology is not fully understood, although cytokine abnormalities suggest that Th1 lymphocytes play an important role in pathogenesis of the dermatosis. Factors inducing Sweet's syndrome include: haematopoietic hyperplasia; neoplasms: genitourinary, breast, gastrointestinal; infections of the respiratory and alimentary system; inflammatory bowel diseases; drugs; pregnancy and vaccinations. Systemic corticosteroids are the "gold standard" of Sweet's syndrome treatment; potassium iodide or colchicine may also be used. Indomethacin, clofazimine, cyclosporine A and sulfones are the second-line drugs.Postepy Dermatologii I Alergologii 02/2014; 31(1):47-52. · 0.66 Impact Factor