Cataract Dermatogenic

Dermatogenic cataract

Dermatogenous cataract (s. dermatogena; synonym: Andogsky syndrome) is a rare form of congenital cataract associated with impaired development of the ectoderm. It is characterized by a combination of bilateral cataracts with changes in the skin and its appendages.

The reason is a mutation in the IP3R1 gene, which encodes the receptor protein for ionotropic purinergic receptors. This protein is involved in signal transmission from purine receptors to intracellular signal transduction pathways that regulate cell proliferation and differentiation.

Clinical manifestations:

1. Bilateral congenital cataracts of varying degrees of density.

2. Skin abnormalities: ichthyosis, atrophic spots, hyperkeratosis.

3. Nail abnormalities: koilonychia, absence of nails.

4. Hair abnormalities: hypotrichosis, atrichosis.

5. Dental malformations.

6. Delayed mental and physical development.

Diagnosis is based on a combination of ophthalmological and dermatological symptoms. Treatment is symptomatic - surgical removal of cataracts, correction of skin and dental defects. The prognosis mainly depends on the degree of developmental delay.

Cataracts are a change in the optical properties of the lens of the eye due to various violations of the structure of its tissue, which leads to deterioration of vision or the development of blindness.

Cataracts are preceded by clouding, which occurs due to various pathological processes in the eye. Sometimes this clouding may have a yellowish or grayish tint. With the development of cataracts, the illumination of the fundus of the eye decreases, and the patient begins to see worse. In advanced cases, the disease leads to complete blindness. Cataracts occur quite rarely in young people, and more often develop in older patients (senile age). This disease can occur either after an illness or after many years. It also sometimes occurs with a general serious illness of the body.

Dermogenic cataract (syn.: Andogi syndrome) is an ophthalmological disease that is characterized by clouding of the lens of the eye caused by the deposition of various substances in it. It usually occurs in people aged 60-70 years.

The underlying cause of this disease is unknown. It is thought that it may be related to genetic factors as well as environmental factors such as air pollution and alcohol consumption. There is also evidence of the possible influence of tobacco smoking on the development of dermogenic cataracts. In the initial stage of development of symptoms, blurred vision is noted, especially with a sharp transition from bright light to a dark room or vice versa. However, later red spots and swelling appear on the skin of the eyelids and face. There is pain and stinging in the eyes. As cataracts progress, vision may deteriorate, especially at dusk or in dim light. Ophthalmological examination reveals a spot of calcium salt deposits on the posterior surface of the cornea. This area is initially transparent and becomes cloudy until opaque dense areas of varying sizes appear. The appearance of spots is promoted by the formation of white calcium deposits in the front of the lens. Treatment consists of prescribing anti-inflammatory drugs, vitamins, physical therapy and vision protectors. If there is a threat of vision loss, surgical interventions are possible aimed at removing calcium deposits and lens tissue with subsequent implantation of an artificial lens.