Skin lesion with a fatal neurologic outcome (Degos' disease)

Degos' disease, or malignant atrophic papulosis, is a rare multisystem vasculopathy of unknown etiology. Kohlmeier ^[1] first reported it in 1941 as a form of thromboangiitis obliterans but in 1942 Degos et al. ^[2] described it as a distinct entity. The clinical hallmark of the disease is a cutaneous eruption on the trunk and extremities that is characterized as umbilicated papules with atrophic ``porcelain white'' centers that are painless but occasionally itchy. ^[3] The pathologic substrate of the disorder is progressive occlusion of middle- and small-sized arteries and veins, culminating in ischemic infarction of skin and viscera. ^[4] Inflammatory changes in the affected vessels are mild and infrequent. Gastrointestinal involvement is the most frequent systemic complication, with intestinal perforation being the most common cause of death. ^[5,6]

Nervous system involvement has included multiple cerebral infarcts, venous sinus thrombosis, subdural hematoma, and, less commonly, vascular cord lesions, myopathy, and neuropathy. ^[5,7-16] The neurologic manifestations of Degos' disease are rarely reported, and only anecdotally. We report the Mayo Clinic experience with the neurologic features of Degos' disease in a series of 15 patients.

Patients and methods.

The medical records of patients who were given the diagnosis of Degos' disease between 1954 and 1993 at Mayo Clinic Rochester and Mayo Clinic Scottsdale were retrospectively reviewed. Fifteen patients were included in the study who had the typical skin lesions of Degos' disease, which was confirmed by skin biopsy Figure 1 and Figure 2. One patient was excluded due to the absence of typical skin lesions. The duration of follow-up (onset of first symptom to last contact with the patient either during a clinic visit or via telephone/letter) ranged from 2 to 27 years (median, 13 years). Nine patients have survived and six patients have died. Autopsy reports of four of …