α 1-Antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, α1-antitrypsin deficiency may be associated with panniculitis. In this article we describe three patients in whom the recognition of certain clinical and histologic features of panniculitis eventually led to the diagnosis of α1-antitrypsin deficiency. Two of our patients were young adults and one was a child. All three had draining, panniculitis, or cellulitis-like lesions at sites of prior trauma. The histopathologic findings were characterized by liquefactive dermal necrosis and collagenolysis of the fibrous septa of the, subcutis. The combination of these clinical and microscopic findings should suggest the diagnosis of α1-antitrypsin deficiency panniculitis. The suspicion can be verified by obtaining quantitative serum levels and enzyme phenotyping. The identification of the arantitrypsin deficiency state as the cause of a distinct type of panniculitis adds additional evidence for the elimination of the term Weber-Christian disease.
ASJC Scopus subject areas