Degos Disease, also known as Malignant Atrophic Papulosis, is a rare and progressive disorder that affects the blood vessels. It primarily affects the skin, but can also involve other organs such as the gastrointestinal tract, central nervous system, and eyes. The exact cause of Degos Disease is unknown, and it predominantly affects adults between the ages of 20 and 50.
Skin Lesions: The most common and characteristic symptom of Degos Disease is the development of skin lesions. These lesions typically start as small, red or purple papules that gradually evolve into raised, firm, and porcelain-white plaques. The lesions have a central depression and a surrounding erythematous rim, giving them a "cayenne pepper" appearance. These skin lesions can occur anywhere on the body but are most commonly found on the trunk, face, and limbs.
Gastrointestinal Involvement: In some cases, Degos Disease can affect the gastrointestinal tract. This can lead to various symptoms such as abdominal pain, nausea, vomiting, diarrhea, and gastrointestinal bleeding. In severe cases, it can cause bowel perforation or obstruction, which requires immediate medical attention.
Central Nervous System Involvement: Degos Disease can also involve the central nervous system, leading to neurological symptoms. These can include headaches, seizures, cognitive impairment, memory loss, difficulty speaking, and changes in behavior. In rare cases, it can cause strokes or transient ischemic attacks (mini-strokes).
Ocular Manifestations: Some individuals with Degos Disease may experience ocular manifestations. These can include inflammation of the eye (uveitis), retinal artery occlusion, or retinal detachment. Ocular involvement can lead to vision loss or blindness if not promptly treated.
Systemic Complications: In advanced stages of Degos Disease, the condition can progress to involve multiple organs, leading to systemic complications. These complications can vary widely and may include problems with the heart, lungs, kidneys, liver, or pancreas. Systemic involvement can significantly impact overall health and may require intensive medical management.
Other Symptoms: Some individuals with Degos Disease may experience general symptoms such as fatigue, weight loss, fever, and muscle or joint pain. These symptoms are non-specific and can be seen in various other conditions, making the diagnosis of Degos Disease challenging.
It is important to note that the symptoms and severity of Degos Disease can vary widely among individuals. Some individuals may have mild skin involvement, while others may experience more severe systemic complications. Early diagnosis and appropriate management are crucial in order to prevent or minimize organ damage and improve overall prognosis.