SKIN INVOLVEMENT IN AMYOTROPHIC-LATERAL-SCLEROSIS

Background Patients with sporadic amyotrophic lateral sclerosis (ALS) show disorganised collagen and elastin of the dermis. We looked for in flammatory alterations to cutaneous blood vessels. Patients and findin gs Seven patients with sporadic ALS were investigated; five were confi ned to bed, but none had bedsores. Light and electron microscopy of sk in showed an oedematous dermis with collagen fibrils of irregular diam eter. Small blood vessels were characterised by duplicated basement me mbranes and deposition of beta-amyloid protein, the main component of the neuronal and non-neuronal amyloid deposits in Alzheimer's disease. These skin changes were seen in all degrees of disability, but none w as found in age-matched and sex-matched controls. Interpretation The s kin in ALS is characterised by a distinctive pattern of alterations of connective tissue and blood vessels. Examination of skin is an additi onal and easily accessible investigation which may help elucidate the pathogenesis of ALS.