Physical Manifestations Of Degos Disease

The main physical finding of Degos Disease is a skin rash that manifests with porcelain white scars. Degos Disease also affects the eyes, the intestines, the brain, and other organs with a variety of physical findings.

• The most visible manifestation of Degos Disease is in the skin.
• Degos Disease manifests with (in most cases) multiple, small, red, raised papules that are 2-5 mm in diameter. After a few days, they enlarge and develop a central white spot that is depressed in comparison to the red skin around it. They heal, leaving depressed porcelain white scars with a rim of telangiectases.
• The papules predominantly occur on the trunk and the arms.
• Degos papules commonly manifest on the penis.
• Degos more rarely occurs on the palms and soles.
• One case has noted that Degos occurred on the scalp.
• No cases known to the author note Degos Disease-type papules on the face.
• In a 17-month-old child, progressive involvement of the fingers and the toes with torpid ulcers and apical necrotic amputations has been reported.
• Peristomal lesions have been reported.
• The rash of Degos Disease develops slowly and is usually mostly asymptomatic, but it may be accompanied by a slight burning sensation. The rash can arise anywhere except on the soles, the palms, and the face. The rash starts as pink or red papules that are 2-15 mm in diameter. The papules evolve into atrophic scars that are porcelain white. Sometimes, abdominal symptoms precede the rash, but this finding is uncommon.
• A variety of ocular findings occur in Degos Disease.
  Posterior subcapsular cataracts, visual field defects, ptosis, third cranial nerve palsies, blepharoptosis, and optic atrophy may be associated with Degos Disease. Optical neuritis, papilledema, and scleral plaques can be present.In 1986, Sibillat et al reported that ophthalmologic symptoms were present in 35 of 105 extant reports of Degos Disease. It manifested in the eye tissues, usually in the conjunctiva. The sclera, the episclera, the retina, the choroid, the optic nerve, and/or the neuro-ophthalmologic apparatus demonstrated damage consonant with Degos Disease.
• Constrictive pericarditis has been reported in Degos Disease. This condition might be induced by pericardial vasculitis, thereby causing the left ventricular wall motion abnormality.
• The lungs can be affected in Degos Disease. Pulmonary manifestations include pleuritis and bilateral pleural effusions.
• The liver and the kidneys may be involved and associated with a vasculitis.
• The brain and the nerves can be affected in Degos Disease. In 1 patient with Degos Disease, the neurologic examination revealed a right-hemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. In another patient, an ascending thoracic myelopathy was present.
  In 1996, at the Mayo Clinic, Subbiah et al described a series of 15 patients. Ten patients developed neurologic manifestations. These findings included fatal hemorrhagic or ischemic strokes, disabling polyradiculoneuropathy, and nonspecific neurologic symptoms without objective findings.
  Other manifestations include strokes, headaches, epilepsy, or nonspecific neurologic symptoms (eg, memory loss, altered sensation).
• Patients experience abdominal pain. Gastrointestinal bleeding can result in vomiting blood or passing blood with bowel movements. Patients with Degos Disease may have enterocutaneous fistulae.