Pasini-Pierini Limited Atrophoscleroderma

Pasini-Pierini is a rare skin disease. The first symptoms usually appear between the ages of 30 and 50, although the disease can develop at any age. The disease is characterized by slowly progressive desquamation of the skin and changes in texture. It is very rare that irreversible changes develop.

Pathological changes in pasini - Pierini affect the epidermis or directly the dermis. Excessive keratinization leads to the formation of islands (papilomatous forms), which further undergo keratinization, which increases the risk of inflammation.

Histologically, Pasini and Pierini

Contents: Pasini – Pierini-limited atrophos-cleroderma

- Pasino-Pierrini - limited atrophask-leroderma. (PPOA) is pathomorphologically characterized by a monochrome lymphoplasmacytic cell infiltrate, consisting predominantly of nummular and dendritic cells, locally containing foamy and hyalinized nuclei, and is located in the subcutaneous fat and dermis. It occurs most often in women, predominantly aged 40-60 years. It manifests itself as a painless, wide, shiny café-au-lait papule; later they group into plaques and merge, gradually disintegrating with the formation of pigment (hethemes) and atrophic scars. Myasthenia gravis begins to develop, and the temperature rises.

In the peripheral blood there is moderate leukocytosis with lymphomonocytosis. Tendon biochemical reactions are sharply positive. The serum content of protein, fibrinogen, hyaluronidase was increased, and hematocrine reactions were positive. Protein-sedimentary reactions with elastase and alpha-1-antitrypsin are sharply positive, and in joint homogenates in the form of foci of demyelination and hemosiderosis. The diagnosis is confirmed by radiation research methods: cyanovimography, immunological and histological research methods. After surgical treatment, patients with PPOA are closely monitored. Drug therapy includes taking antimalarial drugs for hypertensive purposes; for peripheral vascular spasm, ganglion blockers and calcium antagonists are prescribed.

Some patients may develop PPOA after kidney transplantation. For long-term restrictive joint syndrome, bone marrow puncture with preoperative X-ray contrast osteoscintigraphy, hemosorption, and lymphocytapheresis are indicated. Recently, pulsed UHF therapy has proven itself well. The optimal method is microsurgery with complete excision of the pathological focus (like otoplasty) in combination with