Although the exact pathogenesis of LS is still unclear, the recognized active involvement of skin immune system (e.g., activated T cells and CD1a+/HLA-DR+ dendritic cells) and the association with autoimmune disease and human leukocyte antigen DQ7 in women and girls with LS suggests an immunogenetic component to the disease [2,4]. The gene discussed is CD1A; the disease is Leigh syndrome.