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Dermatol Clin. 2008 Oct;26(4):447-51, vi. doi: 10.1016/j.det.2008.05.001.

Alpha-1-antitrypsin deficiency panniculitis.

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  • 1"Hospital Universitario Doce de Octubre", Universidad Complutense, Madrid, Spain.

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.

PMID:
18793976
[PubMed - indexed for MEDLINE]
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