Severe Neurologic Involvement of Degos Disease in a Pediatric Patient

KARAOĞLU P., Topcu Y., Bayram E., YİŞ U., AKARSU S., Atalay E., ...Daha Fazla

JOURNAL OF CHILD NEUROLOGY, cilt.29, sa.4, ss.550-554, 2014 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 29 Sayı: 4
  • Basım Tarihi: 2014
  • Doi Numarası: 10.1177/0883073812474692
  • Dergi Adı: JOURNAL OF CHILD NEUROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.550-554
  • Anahtar Kelimeler: Degos disease, subdural effusion, encephalomalacia, polyradiculoneuropathy, vasculopathy, MALIGNANT ATROPHIC PAPULOSIS, SKIN
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

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 papules with a white atrophic center surrounded by pink rim mostly on the trunk and lower extremities. Brain magnetic resonance imaging showed chronic subdural effusion and encephalomalacia of the left cerebral hemisphere. Cerebrospinal fluid (CSF) examination revealed increased protein levels. Electromyography was consistent with diffuse polyradiculoneuropathy. Skin biopsy confirmed the diagnosis of a rare vasculopathy: Degos disease. A case presenting with chronic subdural effusion, encephalomalacia, elevated CSF protein, and polyradiculopathy should be carefully examined for skin lesions, which may suggest the diagnosis of Degos disease.