^

Health

A
A
A

Familial acrogeria (Gottron syndrome): causes, symptoms, diagnosis, treatment

 
, medical expert
Last reviewed: 07.07.2025
 
Fact-checked
х

All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.

We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.

If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.

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.

trusted-source[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ], [ 7 ]

What do need to examine?

How to examine?

You are reporting a typo in the following text:
Simply click the "Send typo report" button to complete the report. You can also include a comment.