Family acrogeria (Gottron syndrome): causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Family acroheria (Gottron's syndrome) is a rare disease described in 1941 by N. Gottron.
The causes and pathogenesis of the acroheria of the family (Gottron's syndrome) are not fully understood. In the development of the disease, an important role is played by the disruption of the structure and function of fibroblasts and collagen synthesis, hypofunction of the pituitary gland. There are reports of family cases of the disease. Currently, many dermatologists consider acrogeia a family hereditary connective tissue disease that is transmitted by an autosomal recessive type.
Symptoms of acrogeria family (Gottron's syndrome). Foci of 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 (mostly on the nose), chin and ears. The skin of the rear and hands of the feet has an old age: dry, thin, wrinkled (geradermia), yellowish in color, translucent, easily traumatized with the formation of bruises and scars. Thinning of subcutaneous fat in these areas is noted. The fingers are conically thinned, the brushes are small (acromycria), the chin is small; rarely observed dystrophy of the teeth and nails. Hair is usually not changed. Other skin areas are also not changed. In the literature, other anomalies, the presence of foci of poikiloderma, and the underdevelopment of secondary sexual characteristics are described.
The nail plates of the feet are deformed, subjected to onycholysis.
Histopathology. Detect atrophy of the dermis, to a lesser extent - subcutaneous tissue. Collagen fibers are arranged incorrectly, degenerate and the number of them is reduced.
Differential diagnosis should be conducted with acquired geradermia, Chernogubov-Ehlers-Danlo syndrome, children's progeria.
Treatment of acroheria of the family (Gottron's syndrome) is symptomatic. It is necessary to guard against the effects of mechanical and physical factors. Patients recommend drugs that improve trophicity (actovegin, solcoseryl), creams with vitamins A and F.
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