The initial case of Olmsted syndrome was documented by H.C. Olmsted in 1927. This case involved a five-year-old male who developed sharply demarcated palmoplantar keratoderma during his first year of life. The severe keratoderma resulted in flexion deformities and autoamputation of the digits. Additionally, the patient presented with sharply defined, hyperkeratotic plaques around the periorificial regions (Olmsted 1927).