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| Fig. 19.7 Alpha-1 antitrypsin deficiency panniculitis showing foci of hemorrhage in the center of the lesion. (Courtesy of Kenneth E. Greer, MD.) |
Since the mid-1970s, it has become apparent that patients with this inherited proteinase inhibitor deficiency, especially those most severely affected and having the homozygous PiZZ phenotype, are prone to develop painful hemorrhagic subcutaneous nodules (Fig. 19-7) that ulcerate and drain. Without α-1 antitrypsin, the activity of neutrophil elastase is unchecked. It is believed that in such individuals a variety of triggering factors, such as trauma, may initiate a sequence of events that includes unchecked complement activation, inflammation, endothelial cell damage, and tissue injury. Microscopic clues to the diagnosis include diffuse neutrophilic infiltration of the reticular dermis, and liquefactive necrosis of the dermis and the subcutaneous septa, with resultant separation of fat lobules. Treatment options include dapsone, systemic corticosteroids, plasma exchange therapy, and, more recently, parenteral administration of a proteinase inhibitor.
Chowdhury MM, Williams EJ, Morris JS, et al: Severe panniculitis caused by ZZ alpha-1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin), Br J Dermatol 147:1258–1261, 2004.
