Figure 2. Muscle pathology. (H&E: hematoxylin-eosin) (A) Immunohistochemistry shows negative staining of VLCAD in the patient with VLCAD deficiency. (B) In PCD, lipid droplets are markedly increased in both number and size in muscle fibers, especially type 1 fibers. (C) In MADD, similar features of increased lipid accumulation are seen as those in PCD. (D) In NLSDM, except for increased lipid droplets, rimmed vacuoles are seen in muscle fibers.