Panniculitis – Alpha 1 Antitrypsin Deficiency Panniculitis
Alpha 1 Antitrypsin Deficiency Panniculitis is a rare form of panniculitis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Alpha 1 Antitrypsin is an enzyme that normally circulates throughout the body and functions as an anti-protease enzyme. Patients who have a genetic deficiency in Alpha 1 Antitrypsin do not have the proper control of certain protease enzymes such as neutrophil elastase in their body and subsequently develop liver and lung damage. Patients with this deficiency are also prone to developing a panniculitis. This type of panniculitis usually begins after trauma and presents as nodules and a draining oily viscous liquid from openings in the skin that may occur. In patients with normal levels of alpha 1 antitrypsin, the enzymes present in trauma induced inflammation are deactivated before dissolution of the adipose tissue occurs leading to a panniculitis. Treatment involves replacement of the enzyme alpha 1 antitrypsin as well as antibiotics doxycycline and dapsone which inhbit neutrophil chemotaxis. Systemic steroids may worsen the panniculitis.