Acquired cutis laxa secondary to Sweet syndrome in a child (Marshall syndrome): A rare case report

Abstract

Sweet syndrome is rare in the pediatric population and usually responds well to treatment, resolving without sequelae. Marshall syndrome is a rare pediatric skin disease characterized by loss of elastic tissue (cutis laxa) secondary to acquired, localized neutrophilic dermatitis without any internal organ involvement. Only few cases of Marshall syndrome (acquired cutis laxa type II) have been reported. Systemic steroids and dapsone show excellent results in Sweet syndrome. Although there is no satisfactory treatment for cutis laxa, dapsone can be used in the acute phase for control of swelling.

New Practical Aspects of Sweet Syndrome

“…SS usually presents in individuals in their sixth decade of life [7,10,11]. Occurrence of SS in the pediatric population is rare (with an estimated 5-8% of cases of SS being reported in children [12]), although several cases of pediatric SS have been recently reported in literature [13][14][15][16][17][18][19][20]. In a 2019 French study of 10 pediatric SS patients, the investigators reported a median age of 2.7 years at presentation and a female to male ratio of 2:3.…”

Acquired cutis laxa type II (Marshall syndrome) in a 3‐month‐old boy

“…Inherited forms may occur due to mutations in elastin (ELN) , fibulin 5 and 4 , or ATP6V0A2 genes 2 . Type I acquired cutis laxa has an insidious or post‐inflammatory onset in adults, with or without systemic involvement including emphysema, pneumothorax, aortic ectasia, hernias, gastrointestinal, and urinary tract diverticulae 1,2 . Acquired cutis laxa type II or Marshall syndrome is a post‐inflammatory elastolysis typically occurring in infancy or childhood and lacks systemic involvement 1 .…”

“…2 Type I acquired cutis laxa has an insidious or post-inflammatory onset in adults, with or without systemic involvement including emphysema, pneumothorax, aortic ectasia, hernias, gastrointestinal, and urinary tract diverticulae. 1,2 Acquired cutis laxa type II or Marshall syndrome is a post-inflammatory elastolysis typically occurring in infancy or childhood and lacks systemic involvement. 1 It should not be confused with another "Marshall syndrome," an autoinflammatory periodic fever syndrome.…”

“…1,2 Acquired cutis laxa type II or Marshall syndrome is a post-inflammatory elastolysis typically occurring in infancy or childhood and lacks systemic involvement. 1 It should not be confused with another "Marshall syndrome," an autoinflammatory periodic fever syndrome. 3 Acquired cutis laxa type II presents as crops of inflammatory erythematous papules and plaques associated with constitutional symptoms.…”

“…Elastolysis may occur due to elastase released by neutrophils infiltrating the dermis or due to dysfunction of elastase inhibitors, mainly alpha-1 antitrypsin, particularly in patients who have pulmonary involvement. 1,2 Treatment is challenging. Systemic corticosteroids and dapsone can suppress the inflammatory phase, but do not help once elastolysis occurs.…”