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Punctuate porokeratotic keratoderma.

S J Friedman1, P S Herman, M R Pittelkow

  • 1Department of Dermatology, William Beaumont Army Medical Center, El Paso, Tex.

Archives of Dermatology
|November 1, 1988
PubMed
Summary
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Palmoplantar keratotic plugs and pits, termed punctuate porokerotic keratoderma, were observed in two patients. Ultrastructural analysis revealed distinct cellular differences from other porokeratotic conditions.

Area of Science:

  • Dermatology
  • Histopathology
  • Electron Microscopy

Background:

  • Palmoplantar keratotic plugs and pits, clinically described as "music box spine" lesions, present a diagnostic challenge.
  • Understanding the precise histopathological and ultrastructural characteristics is crucial for accurate diagnosis and classification.

Observation:

  • Two unrelated patients presented with chronic (11 and 13 years) palmoplantar keratotic plugs and pits.
  • Histologic examination showed a compact parakeratosis column, similar to porokeratosis cornoid lamella.
  • Ultrastructural analysis of affected skin revealed pyknotic nuclei in the stratum corneum and reduced keratohyalin granules in the stratum granulosum.

Findings:

  • The observed ultrastructural features in the stratum corneum and stratum granulosum were distinct from those typically seen in porokeratosis of Mibelli and disseminated superficial actinic porokeratosis.

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  • These distinct cellular findings support a unique pathological entity.

    • Implications:

    • The findings suggest that "punctuate porokerotic keratoderma" is a distinct nosologic entity within the spectrum of porokeratotic conditions.
    • This designation aids in accurate clinical diagnosis and may guide future research into the specific pathogenesis and treatment of this condition.