کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3196101 1201672 2008 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Alpha-1-Antitrypsin Deficiency Panniculitis
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی امراض پوستی
پیش نمایش صفحه اول مقاله
Alpha-1-Antitrypsin Deficiency Panniculitis
چکیده انگلیسی

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.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Dermatologic Clinics - Volume 26, Issue 4, October 2008, Pages 447–451
نویسندگان
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