PMC4782534_01
Schematic representation of the pattern of sensory loss to pain and temperature (a) and clinical photograph shows bifacial weakness. (b) sural nerve biopsy demonstrates vacuolation of the Schwann cells within the Schwann cell subunits. (c) and mild fiber loss (d) Skin biopsy reveals accumulation of bloated, finely vacuolated foam cells with granular cytoplasm around cutaneous adnexa. (e) the vacuoles contain Oil Red O positive neutral lipidic material. (f) and electron micrograph reveals numerous lipid vacuoles in the cytoplasm of fibroblasts (g)
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F1
Schwann cell cytoplasm indenting the nucleus [Figure 1]. Skin biopsy revealed numerous bloated, finely vacuolated fibroblasts throughout the entire thickness of the skin, surrounding the dermal capillaries, nerve twigs, and cutaneous adnexal structures. The vacuolated granular cytoplasm was seen indenting the nucleus [Figure 1]. These vacuoles stained with Oil Red O, confirming the presence of lipid rich material [Figure 1].