Petja-Kleja Disease

Petzsch-Klebs disease (Petzsch-Klebs Durchblutungsstörungen) arose as a result of the discovery of two separate diseases - Petzsch's disease and Klez's disease, and consists of a disruption of the normal blood supply to tissues, accompanied by inflammatory changes in the nervous tissue. It was named after the Austro-Hungarian dermatologist, professor Peter Petrovich Keza (G. Petzsch, 1943-1914) and the French doctor Eduard Clejat (1789-1860). The skin of the face, especially the cheeks, is usually affected by this disease, but the parietal, sometimes occipital and temporal regions, as well as areas of the scalp are also affected. If the upper parts of the chest or sometimes the thigh and lower legs are affected, then they speak of a spinal form of the disease. Favorite location of the elements of the disease: the lateral halves of the nose, its wings. Hyperemia, infiltration and multiple hemorrhages above it indicate that this is a rigid dermatosis. The characteristic “strawberry” pattern is clearly visible on the reddened skin, visible when it is smeared with iodine tincture. This

**Petja-Klezha Disease**

Petja-Klej disease (Petja-Klej sequence) is a multidisciplinary disease characterized by a deep degree of alopecia and, in the case of the affected fetal head, congenital extensive alopecia. Symptoms begin in early childhood. This type of disease is often called “hourglass disease” because of its resemblance to an hourglass. In the late 16th century, the condition was known and described as “hourglass” due to the presence of early hair loss and the hourglass-like appearance of balding areas of skin. It was not until 1985 that Kenneth N. Bryan coined the term “adult atopic hair loss,” which was replaced by “hourglass disease” in 2008. For the first time the disease was described under this name in the work. Research has shown that genetic factors play an important role in the pathogenesis of hourglass disease. The most common type of baldness in men is linked to the Y chromosome, but is sometimes seen in women 1. A genetic cause of autosomal dominant baldness, in which the allele causes hair loss in the mother and the man. Also known as “congenital hyperorthopherin deficiency.” First identified and described in the work of Robert and Jenny Knapp. The defective protein was found to transport a growth hormone known as ferryltron, which is produced by the thyroid gland, disrupting the transport of pheofibroid hormones, which leads to the differentiation of hair follicle cells.

Although the first symptoms of the disease may appear as early as childhood, the typical age of onset is between 3 and 9 years, they usually appear much later in adolescence. As a rule, this manifests itself primarily in the back of the head (less often on the forehead or upper eyelid). The hair on the back thins less frequently, and after puberty, the follicles on the back can reach full development 2. The condition of the hair worsens with age and becomes permanent. The diagnosis is made by examination through instrumental and laboratory testing, which determines the following symptoms: hair thinning with delayed hair growth; hair fragility up to its complete destruction, followed by the appearance of hairless areas on the scalp; skin atrophy; thinning and brittle nails. There are also changes in pigmentation of the skin and mucous membranes; decreased skin resistance; hypo- and depigmentation (partial or complete) of the skin, eyelashes, eyebrows; swelling of the eyelids; hypertrophy of the skin glands. Possible diagnostic tests are a blood test to determine the level of serum protein (25-OH vitamin D test), a test to detect p-tensin and/or p-factor (vitamin A), a spectrum of abnormalities in endocrine status, etc., but results are poorly specific, vary from laboratory to laboratory, and may even be normal. Risk factor associated with hair growth