Medline ® Abstract for Reference 20
of 'Panniculitis: Recognition and diagnosis'
Alpha-1-antitrypsin deficiency panniculitis.
Valverde R, Rosales B, Ortiz-de Frutos FJ, Rodríguez-Peralto JL, Ortiz-Romero PL
Dermatol Clin. 2008 Oct;26(4):447-51, vi.
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.
"Hospital Universitario Doce de Octubre", Universidad Complutense, Madrid, Spain.