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.

Race, Sex & Age Affected By Degos Disease

Race
Some state that Degos Disease generally occurs in white young adults. However, it is reported in blacks in Africa, Arabs in Jordan, Asians in Japan, and elsewhere. Any racial link is uncertain.

Sex
In 1997, Katz et al noted that the disorder usually occurs in young adults, and the male-to-female ratio is approximately 3:1.
Wilson et al reviewed benign cutaneous MAP in 34 men and women (30 adults and 4 kids) and noted that benign MAP is more commonly reported in women, at a female-to-male ratio of 3:1.

Age
All ages are affected. The fatal systemic variant of Degos Disease can occur in children. In 1999, Lankisch et al described a 16-year-old white adolescent girl with acute abdominal pain due to visceral involvement of Degos Disease that required extensive small-bowel resection. The skin manifestations had been present for 2 years before the correct diagnosis was made. She died as a result of CNS involvement from Degos Disease. Jalil et al described a case in a 2-year-old child who presented with chronic abdominal pain of uncertain origin.

The benign cutaneous variant of Degos Disease can occur in adults. Wilson et al looked at 34 patients with benign cutaneous MAP and found their average age was 37.6 years. In 1998, Farrell et al described a case of a 44-year-old woman with Degos Disease and a lupus anticoagulant who, 4 years later, was alive and without systemic involvement. Electron microscopy of the white papules demonstrated interwoven tubular structures within the endothelial cells. This was consonant with reports in previous studies of DD. Farrell et al thought that aspirin (300 mg/d) kept her cutaneous Degos Disease in check. In 1998, Requena et al described a 58-year-old homosexual man with AIDS who developed typical cutaneous lesions of MAP, with no visceral involvement detected 2 years after the diagnosis of Degos Disease.

Degos Disease can occur in infants. Degos Disease has been reported in a 7-month-old girl who showed spontaneous aggregation of platelets. A good clinical response in this patient was obtained by treatment with aspirin and dipyridamole.

Pathophysiology of Degos Disease

The etiology and the pathophysiology of Degos Disease are unknown। Some have classified Degos Disease as a vasculitis, a mucinosis, or a thrombotic disorder। In most cases, no circulating immune complexes, antiendothelial cell antibodies, or anticardiolipin antibodies are isolated.

Although, in some cases, antiphospholipid antibodies of uncertain significance are identified। Some authorities suggest that Degos Disease involves a primary endothelial cell defect with secondary thrombosis, leading to infarctive changes. No evidence exists for antibodies to components of endothelial cells. Medications and toxic chemicals do not appear to induce Degos Disease.

The actual physical damage to blood vessels involves, at least in part, impaired fibrinolytic activity and alterations in platelet function. Classifying Degos Disease as a vasculitis may not be appropriate because inflammation of the vessel walls is minimal and because immune complexes have not been found in the vessel walls. Three possible mechanisms for this pathology have been suggested: disturbance in immunity, viral infection, and abnormality in the clotting system of blood.

In familial cases, an autosomal dominant mode of inheritance has been suggested, but this is uncertain.

In molecular analysis of cases of Degos Disease with only cutaneous lesions, no paramyxovirus was identified by polymerase chain reaction.

Mortality/Morbidity Of Degos Disease

Systemic DD is frequently fatal within 2-3 years from the onset of systemic involvement. The cause of death is usually intestinal perforation. However, the range of survival time from time of diagnosis varies from less than 1 year to more than 12 years. Other causes of death include bowel infarction, pleuropericardial pathology, and neurologic infarction and hemorrhage.

Wilson et al reviewed the 24 reported instances of MAP malignant/systemic type and benign/purely cutaneous type in pediatric patients. They found that 14 cases (58%) were fatal. Patients died an average of 3.6 years after the diagnosis of MAP.

In 1 patient, in whom skin and abdominal symptoms occurred at the same time, death from bowel hemorrhage followed in 6 मोंथ्स.

In 1996, Subbiah et al described the neurologic features of a series of 15 patients with DD at the Mayo Clinic. Each patient had the white papules that are the hallmark of DD (biopsy proven). Long-term follow-up revealed 6 patients were dead. Nine patients with skin lesions only were nearly asymptomatic. Immunosuppressive and antiplatelet agents did not halt disease progression. CNS infarcts and hemorrhages with intravascular thrombi and without evidence of vasculitis were notable findings at autopsy.

Notash et al reported a 48-year-old Iranian man with lethal systemic DD.

Degos Disease Overview

Degos Disease which is also called malignant atrophic papulosis is a disease named after Robert degos who recognised it as a clinical entity in 1942. It is a very rare vasculopathy results to blockage of vessels and tissue infarction after effecting the inner linning of veins and arteries.

Some ailments like bowel ischemia (mesenteric ischemia or ischemic colitis), chronic skin lesions, ocular lesions, strokes, spinal lesions, mononeuritis multiplex, epilepsy, headaches or cognitive disorders can occur as a result of blood vessels that has been affected;those that can be affected include those supplying the skin,gastrointestinal tract, and central nervous system.

The outcome of this disease can be fatal with a median survival of 2 to 3 years,[1] although some appear to have a benign form (Degos acanthoma) which affects only the skin. There are fewer than fifty living patients at present known worldwide, and less than 200 reported in the medical literature. Treatment options are imited, consist mainly of Antiplatelet drugs or anticoagulants or immunosuppressants, and effect of treatment is limited to case reports.

It has been suggested that this is not a separate disorder, but the final result of several vascular systemic disorders.