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Familial acrogeria (Gottron syndrome): causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
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Acrogeria familialis (Gottron syndrome) is a rare disease described in 1941 by H. Gottron.
The causes and pathogenesis of familial acrogeria (Gottron syndrome) are not fully understood. The development of the disease is largely due to the disruption of the structure and function of fibroblasts and collagen synthesis, and hypofunction of the pituitary gland. There are reports of familial cases of the disease. Currently, many dermatologists consider familial acrogeria to be a hereditary disease of connective tissue, transmitted in an autosomal recessive manner.
Symptoms of familial acrogeria (Gottron syndrome). The lesions appear either at birth or in the first years of a child's life. The disease is more common in females. Unlike other skin atrophies, the pathological process is observed mainly on the hands and feet, less often on the face (mainly on the nose), chin and ears. The skin of the back of the hands and feet has an senile appearance: dry, thin, wrinkled (geroderma), yellowish in color, translucent, easily injured with the formation of bruises and scars. Thinning of the subcutaneous fat is noted in these areas. The fingers are conically thinned, the hands are small (acromicria), the chin is small; dystrophy of the teeth and nails is rarely observed. The hair is usually unchanged. Other areas of the skin are also unchanged. Other anomalies, the presence of poikiloderma foci, underdevelopment of secondary sexual characteristics are described in the literature.
The nail plates of the feet are deformed and subject to onycholysis.
Histopathology. Atrophy of the dermis and, to a lesser extent, subcutaneous tissue is detected. Collagen fibers are irregularly located, degeneratively altered, and their number is reduced.
Differential diagnosis should be made with acquired geroderma, Chernogubov-Ehlers-Danlos syndrome, and childhood progeria.
Treatment of familial acrogeria (Gottron syndrome) is symptomatic. It is necessary to protect against mechanical and physical factors. Patients are recommended drugs that improve trophism (actovegin, solcoseryl), creams with vitamins A and F.
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