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Malignant atrophie papulosis (Kohlmeier-Degos disease) in childhood

From the Department of Pediatrics, Neurology and Pathology, University of Rochester School of Medicine and Dentistry, Rochester, New York.

Malignant atrophie papulosis (Kohlmeier-Degos disease) is reported for the first time with pathologic verification of central nervous system involvement in a child. The disease began in infancy with rare recurring skin lesions; the child died at the age of 7, after progressive neurologic deterioration. Diagnosis was suspected clinically and confirmed by biopsy of a typical skin lesion. Recognition of this disorder by its dermatologie manifestations may obviate invasive diagnostic procedures.

Dr. Homer's address is Department of Pediatrics, Box 631, University of Rochester School of Medicine and Dentistry, 601 Elmwood Ave., Rochester, NY 14642.

Presented at the twenty-sixth annual meeting of the American Academy of Neurology, San Francisco, California, April 1974.

Received for publication July 18, 1975.

This article has been cited by other articles:

				S. Rosemberg, M. B. S. Lopes, M. N. Sotto, and M. S. Graudenz Childhood Degos Disease With Prominent Neurological Symptoms: Report of a Clinicopathological Case J Child Neurol, January 1, 1988; 3(1): 42 - 46. [Abstract] [PDF]