Köhlmeier-Degos Disease (malignant atrophic papulosis) and Neurologic Involvement

Severe Neurologic Involvement of Degos Disease in a Pediatric Patient

“…Chronic recurrent subdural effusion with elevated cerebrospinal fluid protein levels suggests a chronic meningovascular process. 4 Brain magnetic resonance imaging of our patient revealed left cerebral encephalomalacia. During the course of the disease new acute ischemic lesions developed in the central sulcus, brainstem, and cerebellum.…”

“…Neurologic symptoms are rarely the first manifestations of the disease. 4 The first neurologic finding of our patient was recurrent subdural effusion which required catheter replacement for drainage. Subdural fluid collections have been reported in several cases.…”

“…3 Poor prognosis is associated with gastrointestinal tract and neurologic involvement. 4 Reported neurologic features of the disease include cerebral infarcts, subdural effusions, seizures, polyradiculoneuropathy, optic atrophy, and transverse myelitis. 5 The family of our patient did not remember the exact time of the appearance of the skin lesions, but they noticed the lesions nearly 2 months before his intensive care unit admission, after the neurologic symptoms.…”

“…3 However multisystemic disease with gastrointestinal tract and central nervous system involvement has a poor prognosis and usually fatal within 3 years after the onset of systemic involvement. 4 It is most frequently seen in males during the third or fourth decades. 4 Childhood and adolescence Degos disease is rarely reported.…”

“…4 It is most frequently seen in males during the third or fourth decades. 4 Childhood and adolescence Degos disease is rarely reported. 1 We report severe neurologic impairment of Degos disease with recurrent subdural effusions, disabling polyradiculoneuropathy, and cerebral infarcts in an adolescent.…”