Subsequently, fifteen new cases were reported. This entity is distinguished by bilateral palmo-plantar keratoderma and periorificial hyperkeratotic lesions. These two main symptoms constitute characteristic composition of findings, to the exclusion of other syndromes. 7 Other dermatological signs can also be seen in Olmsted syndrome. Previously reported cases had systemic and dermatological signs in addition to the two main diagnostic features.
Some authors believe that hyperkeratotic lesions appear in response to trauma.
The illness usually begins in early childhood, when the child begins to walk and hold things, but Gioral et al have reported a patient with skin lesions appearing at the age of thirty. The first appearance of the disease is palmoplantar keratoderma which begins in the first six months of life. In some patients, periorificial hyperkeratotic plaques may be the first presentation of the disease. This sign usually appears at the age of two. The disease has an insidious and progressive course over many weeks or months.