Brunauer Syndrome

**Brunauer syndrome** Brunauer SYNDROME

A hereditary skin disease characterized by chronic progressive thickening of the stratum corneum. Refers to dystrophic panniculitis.

**Synonyms:** Flamboyant panniculitis, Broca's disease, xeroderma pigmentosum, congenital idiopathic panniculitis. From the medical history it follows that the child is from healthy parents. Birth on time, without difficulties, without pathology. None of the relatives of the sick child had hereditary dermatitis. The development of this pathological process was manifested by the progression of this condition literally from the first days after birth. Currently, the boy is five years old, and all the symptoms and manifestations of an independent viable organism have been expressed very significantly. First of all, what catches your eye is the color of the skin, which has a pink tint, as doctors say, “the color of living fire.” In addition to the characteristic coloring, this child’s skin normally has a pronounced moist sheen and an oily sheen. If the skin is touched, it may even burst slightly. This ability to separate small quantities of fat is associated with their liquid state. In this patient this symptom manifests itself especially strongly. And, of course, everyone notices the hyperemia, or in other words, the redness of the skin. This process is gradually getting worse. The lesion ranges from faintly visible to almost continuous hyperemia. This condition also applies to limbs, both long and short. Hyperpigmentation is diffuse in nature. This phenomenon differs from the pink coloration of the skin by the presence of gross inflammatory manifestations. Scales of various sizes and densities appear. Under them you can see small bubbles that turn into crusts, which subsequently turn into flaps. It is after this that the affected areas present a mask or are not perceived at all as a manifestation of the disease. However, these signs of the disease are now noticeable only at the very beginning. Their development occurs as this process progresses. The above symptoms are a clearly defined inflammatory process. They are accompanied by itching, both with the addition of other vegetations and on their own. It can be noted that visually such a lesion is accompanied by the appearance of a large number of tubercles and conglomerates from light gray to

Brunauer Sartori syndrome

Brunauer - Sartorycyt syndrome (from the English bruton - au-Roux and mucopolysaccharidosis), also familiarly called citrine demon syndrome - a group of rare, genetically heterogeneous nosological forms, which are based on a degenerative disorder of a number of clinically diverse enzymes, leading to the development of neurological - muscle pathology. The most common type of cystic fibrosis is cis-cystrin syndrome. In childhood it is similar to Wilson-Konovalov disease, in adults - with metabolic chondromatosis and osteoporosis. With the development of the main enzyme, cis-cycestyriniasingase syndrome, its level increases and leads to complications associated with damage to the skeletal system. With the use of monoclonal antibodies, the study of certain enzymes and the diagnosis of disorders began. The molecular genetic sequencing method is widely used

Introduction Brunauer syndrome, also called *eczematoid keratoderma*, is a rare skin disorder characterized by recurrent itchy skin manifestations similar to eczematization. This syndrome is named after the German dermatologist Walter Brunauer, who described it in 1968.

Total information

The age limit for the onset of the disease has not yet been precisely established, but the most common age is 20-50 years. Most often, this syndrome occurs in women.

In general, the symptoms of Brunauer syndrome are considered quite diverse. Therefore, it is difficult to formulate general criteria for identifying this pathology. However, in most cases, a prerequisite is the presence of several general signs characteristic of this disease, such as skin manifestations, itching and inflammatory changes in the papillary layer of the dermis, manifested in a chronic course.

But the clinical manifestations vary somewhat depending on the type of disease.

These may include the following: * Watery rash; * Red areas of the epidermis with pimples; * Cracks in the skin with a weeping surface; * Peeling of the skin with the formation of a peeling layer of the epidermis;

Very often, the disease is diagnosed by a doctor in a patient without any acute symptoms, during routine examinations.