Malignant atrophic papulosis (Kohlmeier-Degos disease) in childhood.
Malignant atrophic 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 dermatologic manifestations may obviate invasive diagnostic procedures.
D002490:Central Nervous System / Q000473:pathology*; D002648:Child; D006801:Humans; D008297:Male; D012867:Skin / Q000098:blood supply / Q000473:pathology*; D012871:Skin Diseases / Q000175:diagnosis / Q000473:pathology*; D013927:Thrombosis