Kehlmeyer-Degos Syndrome

Koehlmeyer Deges Syndrome

Koehlmeyer-Deges syndrome (lichenoid arthritis, elephantiasis, ataxic neurosis, asthenopia, ophthalmopathy) is a rare skin disease that manifests as swelling and red or purple spots on the skin caused by enlarged subcutaneous capillaries. The disease may also be accompanied by muscle weakness, loss of coordination, fainting and other symptoms of a nervous disorder.

The syndrome is named after the Swiss pathologist Othmar Kehlmeyer and the French dermatologist Joseph Degos, who first described this type of tumor in 1876. However, the exact causes of this syndrome are still unknown.

Symptoms 1. Skin manifestations are a red or purple rash on the back of the neck, face or scalp. The rash is accompanied by severe itching and pain. The eruptive skin lesions have a lichenoid consistency (distinct tissue similar to fish scales). Lesions can appear on any part of the body and skin. In severe cases, spread throughout the body. The skin becomes swollen and hot.

Kelmeye-degos syndrome is a chronic disease of the circulatory system (arteriopathy), as a result of which critical atherosclerotic occlusion of the coronary arteries gradually develops and post-myocardial cardiosclerosis occurs. Initially, eosinophilic infiltration of the vascular intima appears in the form of increased vessel permeability and intimal thickening