Sweet's syndrome leading to acquired cutis laxa (Marshall's 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 cutis laxa. The cause of this syndrome is unknown. alpha 1-Antitrypsin (alpha 1-AT) deficiency is a codominantly inherited disorder of alpha 1-AT, the major serum antiprotease active against a number of serine-type proteases.


METHODS
The first patient with classic Marshall's syndrome who had coexisting alpha 1-AT deficiency and a review of other cases of Marshall's syndrome are presented, and pathogenic mechanisms are discussed.


CONCLUSIONS
A deficiency of alpha 1-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 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 deficiency.
Hwang
S T
ST
Department of Dermatology, University of California-San Francisco, USA.
Williams
M L
ML
McCalmont
T H
TH
Frieden
I J
IJ
D003483:Cutis Laxa / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D015842:Serine Proteinase Inhibitors / Q000172:deficiency*; D016463:Sweet Syndrome / Q000150:complications*; D013577:Syndrome; D019896:alpha 1-Antitrypsin Deficiency
D015842:Serine Proteinase Inhibitors