ECM1 and Leigh syndrome: Lichen sclerosus is a chronic lymphocyte-mediated inflammatory skin disease, for which increasing evidence suggests an underlying autoimmune mechanism mainly in female genetically predisposed patients with HLA-DQ7 antigen; in fact antibodies against the basement membrane zone, chiefly Binding Protein (BP)180 and BP 230, have been found in 30% of sera of patients with LS, and a high proportion of patients (up to 80%) have specific antibodies targeting extracellular matrix protein-1 [9].