ALPHA-1 ANTITRYPSIN DEFICIENCY PANNICULITIS
By Gary M. White, MD
Alpha-1 antitrypsin deficiency (AATD) can rarely lead to panniculitis. This condition should be suspected in the setting of:
- Recurrent ulcerative subcutaneous nodules or plaques with serosanguineous drainage.
- Lesions presenting primarily on the trunk and proximal extremities.
- Systemic findings of shortness of breath or limb swelling.
- It may occur at any age, but is most common between 30 and 60 years of age.
- Common systemic findings include pulmonary disease and cirrhosis.
Nodules may appear anywhere on the body, although most often on the lower trunk and proximal extremities. Ulceration and drainage may occur.
First-line treatment options for AATD panniculitis include oral dapsone 50–150 mg daily and oral doxycycline 100–200 mg daily. The most successful treatment is likely to be weekly intravenous alpha1-proteinase inhibitor (Prolastin 60 mg kg−1).
A 16-year-old male with a 3-month history of tender, erythematous, subcutaneous nodules affecting his lower legs. He was otherwise systemically well. Alpha-1 antitrypsin levels were very low at a level of 0.36 g/L (normal, 1.10-2.10 g/L). [JAAD 2011;65;227–229].
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