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March 1961

Generalized Scleroderma in Children: Acrosclerotic Type

Author Affiliations


Fellow in Dermatology, Mayo Foundation (Dr. Jaffe); Section of Dermatology (Dr. Winkelmann); Mayo Clinic and Mayo Foundation.

Arch Dermatol. 1961;83(3):402-413. doi:10.1001/archderm.1961.01580090052005

Scleroderma in children is uncommon, and while localized cutaneous forms of the disease occur more frequently among them, cases of generalized scleroderma are especially rare. Two clinical variants of generalized scleroderma have been described: diffuse scleroderma and acrosclerosis.1 Acrosclerosis was first described by Hutchinson,2 and Sellei3 championed its status as a disease sui generis. O'Leary and Waisman4 stressed the importance of vasomotor abnormalities in acrosclerosis and its good prognosis as compared to that of diffuse scleroderma. Acrosclerosis involves predominantly the skin of the face, upper part of the chest, hands, forearms, and occasionally the feet. A vasospastic (Raynaud's) phenomenon is associated with the sclerosis. Varying degrees of systemic involvement may occur, the gastrointestinal, pulmonary, and cardiorenal systems being most frequently affected.

The early dermatologic literature pertaining to scleroderma5-8 in children is confusing because many cases presented as diffuse scleroderma would be, in our opinion, more

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