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malignant atrophic papulosis/astenia
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Papulosis atrophicans maligna (Köhlmeier-Degos disease): a disseminated occlusive vasculopathy.
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Malignant atrophic papulosis usually presents as pathognomonic skin lesions followed by acute abdominal pain, bowel perforation, peritonitis, and death. Rare patients who may lack gastrointestinal symptoms present with central nervous system manifestations, including headache, paresthesias,
Severe neurologic involvement of Degos disease in a pediatric patient.
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A 14-year-old male presented with paresthesias on the right upper and lower extremities, headache, and vomiting. In addition to worsening paresthesia and weakness on the right side of his body, blurred vision, fever, and skin lesions developed. He also had skin lesions characterized with 3-10 mm
CNS involvement in malignant atrophic papulosis (Kohlmeier-Degos disease): vasculopathy and coagulopathy.
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The clinical features in a 42 year old man with malignant atrophic papulosis with CNS involvement are described. They included mental dysfunction, paraesthesiae, weakness of left limbs, with pyramidal tract signs, bilateral ptosis, progressing to total ophthalmoplegia and obtundation. There were two
Infantile neurological Degos disease.
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Degos disease, or malignant atrophic papulosis, is a rare vasculopathy of uncertain aetiology manifesting as a primary dermatological disorder in most cases, but with widespread systemic involvement developing in an undefined proportion of patients. Reported neurological features of Degos disease
[An autopsy case of Degos disease with neurological symptoms--neuropathological observations and increased platelet aggregation].
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We reported clinical and neuropathological observations of a 41-year-old man with Degos disease. He first noted painless skin lesions over the upper extremities in January, 1982. Three years later he was diagnosed as Degos disease by skin biopsy, and treatment with aspirin was started. In September,
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