Filatov-Dukes Disease

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Filatov-Duks's disease (nose-n-Duksea or Filatov-Dukson's disease) is a rare hereditary disease manifested by deformation of the nasal septum and accompanying inflammatory processes in the upper respiratory tract. This disease was first described in 1931 by the Soviet pediatrician N.F. Filatov as the cause of severe inflammatory diseases of the nose and paranasal sinuses after surgery on the septum. Since then, little has been reported about the disease from other scientists.

Symptoms

The patient's nose is constantly inflamed and suffers from constant problems with mucous membranes and pus, which can also lead to long-term pain in the ears. Patients suffer from increased headaches, nasal congestion, runny nose (a symptom of nasal skin), difficulty breathing through the nose, problems with hearing, mouth and chronic fatigue.

**N.F. Filatov studied the causes of nasal diseases and in the course of his research discovered a connection between a progressive nose, the cause of which is a cicatricial narrowing of the passage at the nasal opening as a result of the development of osteomelitis, and nasal deformation. Confirmation of the theory helped to discover that the named disease appears as a result of heredity - a child from a family where there is such a disease has a high risk of its occurrence due to the inheritance of negative traits.**

It is a genetically determined immunodeficiency combined with infiltration of the skin and lymph nodes of the body. It manifests itself in childhood with cytopenias, vasculitis, and degenerative changes in internal organs. Symptoms: conjunctivitis, photophobia, cataracts, damage to the renal tubules in the form of dysuria, polyuria, anuria with oligoanuria. Diagnosis: molecular genetic analysis, including a search for mutations in genes encoding interferon receptors, analysis of sequencing variations of the same genes, determination of active forms of interferons. Treatment includes adequate doses of interferon suppositories, avoidance of contact with other people, and vaccination. Prognosis: may be favorable with timely treatment. In the most severe cases, the disease can be fatal.

In modern medicine, Filatova-Durksa is usually considered as a differentiated skin rash represented by a bilateral red macula with a central inflammatory papulosquamous element. An erythematous, sharply circumscribed pink or slightly hyperpigmented papule with a diameter of a pea to 2–3 cm, which disappears fairly quickly and may be accompanied by itching or burning. On reddened, swollen skin, one or more single, weakly limited petechial reactions with a raised rim, surrounded by follicular rashes, appear. Typical localization is the flexor surfaces of the arms, extensor surfaces of the limbs, torso, interscapular region and buttocks[1]. Filatov-Dukes disease occurs in older children and young adults, but in rare cases even newborns and adolescents under 15 years of age are susceptible to its development. The incubation period usually lasts less than a week; acute