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:

Clinical

Nodules may appear anywhere on the body, although most often on the lower trunk and proximal extremities. Ulceration and drainage may occur.

Treatment

First-line treatment options for AATD panniculitis include oral dapsone 50ā€“150 mg daily and oral doxycycline 100ā€“200 mg daily. The most successful treatment is likely to be weekly intravenous alpha1-proteinase inhibitor (Prolastin 60 mg kgāˆ’1).

References

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;227ā€“229].

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