Source of Support: None, Conflict of Interest: None
Olmsted syndrome is a rare congential palmoplantar keratoderma with progressive periorificial hyperkeratotic lesions, alopecia areata, deafness, lental and nail changes. Only a few cases of this syndrome have been reported so far. Our case is a seven year old boy with massive palmoplantar keratoderma, perianal and periorifical hyperkeratotic plaques and autoamputation of digits. Alopecia areata, hyperkeratosis of elbows and knees and nail changes were also present. His physical and mental developments were normal. Routine laboratory tests were normal. The case is being reported for its rarity.