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Childhood Köhlmeier-Degos disease with atypical skin lesions

Departments of Pathology (Neuropathology) and Neurology, Children's Hospital Medical Center and the Departmentsof Pathology and Neurology, Harvard Medical School, Boston, MA.

We report clinical and pathologic findings in a 16-year-old boy whose disease began in infancy with maculopapular skin lesions, followed by cyclic nodular cutaneous eruptions, intermittent enlargement of liver and spleen, episodic abdominal pain, and sporadic unexplained fever. Subsequently, various ophthalmologic disturbances, along with a multitude of neurologic signs and symptoms, dominated the clinical picture. The CNS bore the brunt of pathologic changes, characterized by widespread leptomeningeal fibrosis, ventricular enlargement, and multiple brain infarcts. Striking intimal thickening led to narrowing or occlusion of almost all the medium-sized and small extraparenchymal arteries.

Address correspondence and reprint requests to Dr. Sotrel, Department of Neuropathology, The Children's Hospital Medical Center, 300 Longwood Avenue, Boston, MA 02115.

Accepted for publication January 6, 1983.

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