In most cases, the initial symptoms of Degos disease are distinct skin Lesions or a rash. Affected individuals develop small elevated Bumps or spots (papules) of varying shape, usually on the trunk and upper arms and upper legs. Initially, only a few Lesions may be apparent. Eventually, 10-40 Lesions may slowly develop and, in some cases, hundreds may develop. The palms, soles, and face are usually spared. The Lesions may sometimes itch (pruritis). The Lesions start as reddish or pink Bumps and eventually the center degenerates (atrophies) such that older Lesions have a reddish border with porcelain-white, atrophic centers.
In certain individuals, blood vessels in areas other than the skin eventually become involved which can result in serious complications. The specific organ systems affected and the severity of associated symptoms can vary greatly. Individuals who develop systemic Degos disease will not develop all of the symptoms discussed below.
The most common area affected by Degos disease outside of the skin is the gastrointestinal tract. Gastrointestinal involvement can occur anywhere from a few weeks to a few years after the skin Lesions develop. In extremely rare cases, gastrointestinal involvement may precede the development of skin lesions.
When Lesions form in the small intestines, they may cause abdominal pain, cramping, nausea, vomiting, diarrhea, bloating, constipation, and the passing of blood with bowel movements or Vomiting up of blood. Some affected individuals may experience weakness, Fatigue and Weight loss from malabsorption. The intestinal Lesions can tear or rupture (perforate) causing the contents of the intestines to leak into the abdominal cavity. This results in Inflammation of the membranes lining the abdominal cavity (peritonitis), a life-threatening complication.
Some individuals with systemic Degos disease experience involvement of the central nervous system (CNS). Symptoms of CNS involvement vary depending upon the specific areas affected, but may include headaches, dizziness, seizures, Paralysis (palsy) of cranial nerves, Weakness of one side of the body (hemiparesis), strokes, and damage to small areas of cells in the brain due to blocked arteries (cerebral infarcts.) Nonspecific neurological symptoms such as memory loss or altered sensations may also occur.
Additional organ systems that can become involved include the eyes, heart, lungs, and kidneys. When the eyes are affected, individuals may develop double vision (diplopia), drooping of the eyelids (ptosis), clouding of the lenses of the eyes (cataracts), atrophy of the optic nerve, Swelling of the optic nerve (papilledema), and loss of part of the field of vision (visual-field defects).
When the heart is affected, individuals may develop weakness, shortness of breath upon exertion, and chest pain. In some cases, Inflammation of the sac-like membrane surrounding the heart (pericardium) may occur. This may develop into permanent thickening, with resulting scarring and contracture of the pericardium (constrictive pericarditis).
Inflammation of the membranes lining the lungs (pleuritis) and fluid collection around the lungs (pleural effusion) has also been reported.