SWEETS-SYNDROME LEADING TO ACQUIRED CUTIS LAXA (MARSHALLS SYNDROME) IN AN INFANT WITH ALPHA(1)-ANTITRYPSIN DEFICIENCY

Background: Marshall's syndrome is a rare pediatric skin disease that is characterized by acquired, localized neutrophilic dermatitis (Sweet 's disease), followed by loss of elastic tissue in the dermis and cuti s laxa. The cause of this syndrome is unknown. alpha(1)-Antitrypsin (a lpha(1)-AT) deficiency is a codominantly inherited disorder of alpha(1 )-AT, the major serum antiprotease active against a number of serine-t ype proteases. Observations: The first patient with classic Marshall's syndrome who had coexisting alpha(1)-AT deficiency and a review of ot her cases of Marshall's syndrome are presented, and pathogenic mechani sms are discussed. Conclusions: A deficiency of alpha(1)-AT may allow proteases such as neutrophil elastase to destroy dermal elastin and, t hus, produce cutis laxa in Marshall's syndrome. Other cases of acquire d cutis laxa should be screened for alpha(1)-AT deficiency to further evaluate this association and to enable patients and their families to be counseled about possible systemic complications of alpha(1)-AT def iciency.