Nine tumors of squamous cell carcinoma developed in a 61-year-old Japanese woman with linear porokeratosis. She had disseminated superficial porokeratosis, but a linear arrangement of pronounced lesions was found only on the left side of the body, and all of the tumors arose on the linear lesions on the left side of the body. Some of her family members had disseminated superficial porokeratosis. This case may represent a type 2 segmental manifestation of disseminated superficial porokeratosis showing a systematized pattern of involvement and pronounced cancer proneness.
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