Central nervous system - Seizure disorders may occur. This is believed to be caused by increased alpha and beta globulin production. A characteristic finding is bean-shaped calcifications within the hippocampus. This is seen on x-ray and CT scan of the brain. Skin biopsy may be performed to help confirm the diagnosis.
Early lesions show eosinophilic hyaline thickening of papillary dermal capillaries.
Later hyperkeratosis is present and the papillary dermis is widened by hyaline material arranged perpendicular to the basement membrane. Hyaline deposits may be seen arranged around hair follicles, eccrine glands, sebaceous glands, arrector pili muscles and nerves. The hyaline material is PAS-positive and diastase resistant. There is no cure. The disease runs a fluctuating course and no therapy is available.