Alpha1‐Antitrypsin Deficiency‐Associated Panniculitis: Case Report and Review of the Literature

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Abstract
Alpha1‐antitrypsin is the primary serum proteinase inhibitor. Alpha1‐antitrypsin deficiency, especially the ZZ genotype, has been linked mainly to emphysema and cirrhosis; it is also associated with panniculitis. A case of α‐AT‐associated panniculitis was documented in a 13‐year‐old girl in whom a deficiency of the enzyme was known to be present from infancy. This is unusual, since α‐AT panniculitis previously was described in older patients without prior knowledge of the duration of the deficiency. Our patient developed erythematous, subcutaneous nodules subsequent to trauma, which later developed into deep, painless ulcers. We report this case so that the condition may be suspected in patients with panniculitis. The diagnosis may be confirmed by measuring quantitative α‐AT serum levels and by enzyme genotyping. The treatment of choice is dapsone.