KRABBE GLOBOID-CELL LEUKODYSTROPHY - ELECTRON-MICROSCOPY SHOWS CHARACTERISTIC INCLUSIONS IN ECCRINE SWEAT GLANDS

Evidence for storage in eccrine sweat glands from skin biopsies of 7 patients with Krabbe globoid cell leukodystrophy is demonstrated. Clear cells showed characteristic free or intravacuolar inclusions containing needle-like and splinter-shaped structures with sometimes clearly outlined paracrystalline-like geometric configurations. These inclusions were very similar to those observed in the characteristic globoid cells found in the central nervous system and in Schwann cells of cutaneous, conjunctival and peripheral nerves. This finding suggests that the storage is not restricted to the nervous system. The diagnostic value of skin biopsy in Krabbe globoid cell leukodystrophy is emphasized.