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Gottron Syndrome

Abstract

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Synonyms of Gottron Syndrome

acrogeria, Gottron type
familial acrogeria
familial acromicria
H. Gottron's syndrome

Disorder Subdivisions

No subdivisions found.

General Discussion

Gottron syndrome (GS) is an extremely rare inherited disorder characterized by the appearance of premature aging (progeria), especially in the form of unusually fragile, thin skin on the hands and feet (distal extremities). GS is described as a mild, nonprogressive, congenital form of skin atrophy due to the loss of the fatty tissue directly under the skin (subcutaneous atrophy). Other findings may include abnormally small hands and feet with unusually prominent veins on the chest; small stature; and/or abnormally small jaw (micrognathia).

Other characteristics that develop later in life may include premature senility, endocrine disturbances and cataracts. Gottron syndrome is thought to be inherited as an autosomal recessive genetic trait. Only about 40 cases have been reported in the medical literature.

There is some debate in the literature regarding a possible relationship between Gottron syndrome and Ehlers-Danlos syndrome, type IV. Some clinicians believe the terms are synonymous. Others disagree.

Organizations related to Gottron Syndrome

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