There has been no reported pathologic abnormality outside the central
nervous system in patients with Unverricht-Lundborg disease (ULD). We
report membrane-bound vacuoles with clear contents in eccrine clear ce
lls and dark cells in five of seven patients with ULD, as well as in o
ne clinically unaffected sibling. Vacuoles were not seen in the biopsi
es of two patients and of eight controls with Lafora's disease. These
findings, though not entirely specific, suggest that skin biopsy may s
erve as a diagnostic aid to give supportive evidence for ULD.