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October 1995

Sweet's Syndrome Leading to Acquired Cutis Laxa (Marshall's Syndrome) in an Infant With a1-Antitrypsin Deficiency

Author Affiliations

From the Departments of Dermatology (Drs Hwang, Williams, McCalmont, and Frieden), Pediatrics (Drs Williams and Frieden), Pathology (Dr McCalmont), and Anatomy (Dr Hwang), University of California-San Francisco.

Arch Dermatol. 1995;131(10):1175-1177. doi:10.1001/archderm.1995.01690220081015

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 cutis laxa. The cause of this syndrome is unknown. α1-Antitrypsin (α1-AT) deficiency is a codominantly inherited disorder of α1-AT, the major serum antiprotease active against a number of serine-type proteases.

Observations:  The first patient with classic Marshall's syndrome who had coexisting α1-AT deficiency and a review of other cases of Marshall's syndrome are pre- sented, and pathogenic mechanisms are discussed.

Conclusions:  A deficiency of α1-AT may allow proteases such as neutrophil elastase to destroy dermal elastin and, thus, produce cutis laxa in Marshall's syndrome. Other cases of acquired cutis laxa should be screened for α1-AT deficiency to further evaluate this association and to enable patients and their families to be counseled about possible systemic complications of α1-AT deficiency.(Arch Dermatol. 1995;131:1175-1177)

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