Two unrelated patients had numerous palmoplantar "music box spine" keratotic plugs and pits of 11 and 13 years' duration. Histologic examination revealed a compact column of parakeratosis resembling that of a cornoid lamella of porokeratotic conditions. Ultrastructurally in clinically affected skin, the stratum corneum contained numerous variable-sized pyknotic nuclei, and cells in the stratum granulosum contained fewer keratohyalin granules. The ultrastructural findings differed from those of porokeratosis of Mibelli and disseminated superficial actinic porokeratosis. The proper nosologic designation should be punctuate porokeratotic keratoderma.