Glycogenic acanthosis is a benign epithelial proliferation characterized by acanthosis and cytoplasmic pallor of squamous epithelial cells secondary to glycogen accumulation. It most commonly occurs in the esophagus, whereas involvement of the oral mucosa, particularly the lip, is rare. Oral lesions may clinically mimic leukoplakia or other white mucosal lesions. Microscopically, the affected epithelium shows well-demarcated acanthosis with pale, glycogen-rich keratinocytes predominantly involving the spinous layer, generally with preserved epithelial maturation and without significant cytologic atypia or inflammation. The pale epithelial cells demonstrate strong PAS positivity with loss of staining following diastase digestion, confirming the presence of intracytoplasmic glycogen. Small solitary lesions may be termed glycogenic acanthoma. Although esophageal glycogenic acanthosis has been described in association with Cowden syndrome, a similar association has not been established for isolated oral or lip lesions.
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