Alpha-1-antitrypsin deficiency panniculitis |
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Authors: | Valverde Ricardo Rosales Belén Ortiz-de Frutos Francisco Javier Rodríguez-Peralto José Luis Ortiz-Romero Pablo L |
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Affiliation: | "Hospital Universitario Doce de Octubre", Universidad Complutense, Madrid, Spain. |
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Abstract: | Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity. Clinical manifestations typically consist of wide nodular lesions on the trunk and proximal extremities that evolve to ulceration and drainage. Histopathologically it presents as a mixed septal-lobular panniculitis pattern with some typical findings referred. Differential diagnosis from other types of panniculitis and neutrophilic dermatosis must be established. Different treatments, including tetracyclines, dapsone, and alpha-1-antitrypsin repositioning, have shown variable efficacy in controlling this disease. |
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Keywords: | Alpha-1-antitrypsin deficiency Panniculitis Neutrophilic panniculitis |
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