Prognosis is poor when systemic involvement is found, but seems to be good in benign forms.
Gastrointestinal involvement may occur in up to 60% of cases – death may occur.
Patients with skin lesions only may have a good prognosis.
Approximately 15% of Degos’ disease exhibit long-term survival with disease often limited to the skin and with no history of catastrophic bowel or central nervous system involvement.
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.
In 1 patient, in whom skin and abdominal symptoms occurred at the same time, death from bowel hemorrhage followed in 6 months.
In 1996, Subbiah et al described the neurologic features of a series of 15 patients with DD at the Mayo Clinic. All 15 patients had the typical skin lesions of DD, confirmed by skin biopsy. Long-term follow-up revealed death in 6 patients; 9 patients were nearly asymptomatic. Immunosuppressive and antiplatelet agents were of no benefit. CNS infarcts and hemorrhages with intravascular thrombi and without evidence of vasculitis were characteristic features at autopsy.
Prognosis Findings at 1st International Meeting on Degos Disease, March 18th-19th 2005
Patients with positive family history had no systemic involvement (ie no lethal outcome from Degos).
In the current study the mean age is 35.4 years.
A graph showed that 85-90% of patients who develop systemic involvement will do that within the first 6 years after the skin lesions appear. (However one patient is known who developed systemic involvement after 13 years). Statistics from our group of 20 patients show the same distribution of probabilities.
Almost 85% of those with systemic involvement developed their symptoms within the first three years after the appearance of the skin lesions.
There is no standard prognosis for patients with systemic involvement; each patient has to be examined separately.
It is possible to have GI involvement and not have any symptoms.
To discover this, patients would need to be monitored regularly, and many patients’ symptoms and data examined.
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