| Alpha-1-antitrypsin deficiency panniculitis. | |
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MedLine Citation:
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PMID: 18793976 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Ricardo Valverde; Belén Rosales; Francisco Javier Ortiz-de Frutos; José Luis Rodríguez-Peralto; Pablo L Ortiz-Romero |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Dermatologic clinics Volume: 26 ISSN: 0733-8635 ISO Abbreviation: Dermatol Clin Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-09-16 Completed Date: 2008-12-09 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8300886 Medline TA: Dermatol Clin Country: United States |
Other Details:
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Languages: eng Pagination: 447-51, vi Citation Subset: IM |
Affiliation:
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"Hospital Universitario Doce de Octubre", Universidad Complutense, Madrid, Spain. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Diagnosis, Differential Humans Panniculitis / diagnosis, etiology*, metabolism Phenotype Skin / pathology* alpha 1-Antitrypsin / genetics, metabolism* alpha 1-Antitrypsin Deficiency / complications*, genetics, metabolism |
| Chemical | |
Reg. No./Substance:
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0/SERPINA1 protein, human; 0/alpha 1-Antitrypsin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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