Degosa Syndrome

disease, Langevin-Degos syndrome) is a rare systemic disease characterized by damage to the blood vessels. The disease was first described by French dermatologists Robert Degos and Maurice Langevin in 1942.

Degos syndrome usually appears between the ages of 20 and 50 and affects various organs and systems in the body, including the skin, central nervous system, heart, digestive system and kidneys. The main symptom of the disease is characteristic skin changes in the form of multiple necrotic scars, which often occur on the skin of the extremities, face and torso.

In addition to skin manifestations, degosa syndrome can cause various symptoms depending on the organs affected. Some patients may experience headaches, dizziness, seizures, and changes in vision due to damage to the central nervous system. The disease can also lead to heart problems such as arrhythmia and coronary artery obstruction, as well as digestive problems and kidney failure.

The causes of degos syndrome are still not fully understood. The disease is believed to be immunological in nature and is associated with dysfunction of the endothelium (the inner layer of blood vessels), which leads to the formation of blood clots in small blood vessels. However, the exact mechanisms of disease development require further research.

Degos syndrome is a rare disease and is often diagnosed late due to its rarity and variability of symptoms. Although there is no specific treatment for the disease, many patients receive symptomatic therapy to relieve symptoms and prevent complications. In some cases, surgery may be required to treat complications such as intestinal obstruction or coronary artery obstruction.

In conclusion, degosa syndrome is a rare and serious disease that affects various organs and systems in the body. The disease requires further research to understand its causes and develop more effective treatments.

Degoza syndrome is a hereditary myeloproliferative disease with decreased platelet levels and bone marrow depression. There is insufficiency of thrombopoietin synthesis.

The diagnosis can be made if the myelogram shows: 1. Anemia with a hemoglobin level greater than 30 g/l. 2. Increase in MCHC level above 34%. 3. Reticulocyte volume from 90 to 280 mm3. 4. Myelogram demonstrates minimal or absent megakaryocytes. 5. In the peripheral blood there are signs of myelofibrolysis and various forms of eosinophilic infiltration of the bone marrow (eosinophilic lesions > 8% of all bone marrow cells). 6. The patient’s age is up to 60 years. Treatment should include allolymphocyte stem cell transplantation or hematopoietic stem cell transplantation