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The diagnosis of Weber-Christian disease was made by clinical and histopathologic findings in a 25-year-old woman who had recurrent nodules on the legs and arms. The patient's history also disclosed multiple episodes of swelling trauma. Histopathologic examination of the lesions showed a prominent
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Granzyme M (GM) is a novel serine protease whose expression is highly restricted to natural killer (NK) cells, CD3(+)CD56(+) T cells, and gamma delta T cells. Using a GM-specific monoclonal antibody, we analyzed the expression of GM in 214 mature T-cell and NK-cell lymphomas. GM was preferentially
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BACKGROUND
Alpha-1-antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha-1-antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant
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Genetically determined deficiency of alpha-1-antitrypsin, the primary serum protease inhibitor, can result in pulmonary emphysema or liver cirrhosis, and panniculitis may be an early symptom. A case of recurring panniculitis in a 29 year-old woman is described, which led to the diagnosis of severe
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Three patients (a 71-year-old man and 2 women, 73 and 50 years, respectively) with recurrent panniculitis associated with alpha 1-antitrypsin deficiency are presented. Because the concept of chronic and exaggerated inflammatory response in the patients with alpha 1-antitrypsin deficiency is based on
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Alpha 1-Antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha 1-antitrypsin deficiency may be associated with panniculitis. In this article we describe three patients in whom the recognition of certain
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TAFRO syndrome is rare, and its pathophysiology remains unclear. We herein report the case of a 66-year-old man who presented at our emergency department with epigastric pain. Contrast-enhanced computed tomography (CT) showed high-density retroperitoneal panniculus with contrast enhancement. He was
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The lipodystrophies are rare disorders characterized by selective but variable loss of adipose tissue. Metabolic complications, such as insulin resistance, diabetes mellitus, hypertriglyceridemia, and fatty liver, increase in severity with the extent of fat loss. The lipodystrophies can be
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Alpha1-antitrypsin deficiency is a genetic disorder which contributes to the development of chronic obstructive pulmonary disease, bronchiectasis, liver cirrhosis and panniculitis. The discovery of alpha1-antitrypsin and its function as an antiprotease led to the protease-antiprotease hypothesis,
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SERPINA1, α-antitrypsin (AAT) is an acute phase protein, a member of the serpin (serine protease inhibitor) super family and one of the most abundant protease inhibitors in the circulation. The clinical importance of AAT is emphasized in persons with inherited AAT deficiency who exhibit high risk of
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