A 28 year old woman came with complaint of one episode of seizure one day back. On examination, there were acneiform scars on the extensor aspect of her arm and bead-like bumps lining the upper and lower eyelid. The dermatologists diagnosed the patient as having lipoid proteinosis. An MRI was performed to rule out PRES
The MRI of the brain shows hypointense symmetrical lesions in both the amygdala, suggestive of calcifications.
Urbach-Wiethe disease is named after Austrian scientists Erich Urbach and Camillo Wiethe, who first described it in 1929. Urbach was an allergologist and dermatologist, while Wiethe was an otologist.