Pathological characterization of pachydermia in pachydermoperiostosis

Keiji Tanese, Hironori Niizeki, Atsuhito Seki, Atsushi Otsuka, Kenji Kabashima, Keisuke Kosaki, Masamitsu Kuwahara, Shun Ichi Miyakawa, Mikiko Miyasaka, Kentaro Matsuoka, Torayuki Okuyama, Aiko Shiohama, Takashi Sasaki, Jun Kudoh, Masayuki Amagai, Akira Ishiko

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10 Citations (Scopus)

Abstract

Pachydermoperiostosis is a rare hereditary disease, which presents with the cutaneous manifestations of pachydermia and cutis verticis gyrata. Histological findings in pachydermia frequently include dermal edema, mucin deposition, elastic fiber degeneration, dermal fibrosis and adnexal hyperplasia. However, the severity of these findings varies between clinical reports, and a systematic multiple-case clinicopathological correlative analysis has not been performed to date. In the present study, we reviewed the skin biopsy specimens obtained from the pachydermia of six pachydermoperiostosis patients. The severity of the characteristic histological features was semiquantitatively evaluated and correlated with the grade of pachydermia. Dermal edema, mucin deposition and elastic fiber degeneration were observed in all cases. Patients with severe pachydermia had sebaceous gland hyperplasia and fibrosis. These results suggest that the triad of mucin deposition, dermal edema and elastic fiber degeneration are found from very early stage pachydermia, and could be considered diagnostic findings. To ensure an earlier diagnosis of pachydermoperiostosis, a biopsy should be taken when a patient has grade 1 pachydermia to determine the presence of this histological triad.

Original languageEnglish
Pages (from-to)710-714
Number of pages5
JournalJournal of Dermatology
Volume42
Issue number7
DOIs
Publication statusPublished - 2015 Jul 1

Keywords

  • dermal edema
  • elastic fiber degeneration
  • fibrosis
  • mucin deposition
  • pachydermoperiostosis
  • sebaceous hyperplasia

ASJC Scopus subject areas

  • Dermatology

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  • Cite this

    Tanese, K., Niizeki, H., Seki, A., Otsuka, A., Kabashima, K., Kosaki, K., Kuwahara, M., Miyakawa, S. I., Miyasaka, M., Matsuoka, K., Okuyama, T., Shiohama, A., Sasaki, T., Kudoh, J., Amagai, M., & Ishiko, A. (2015). Pathological characterization of pachydermia in pachydermoperiostosis. Journal of Dermatology, 42(7), 710-714. https://doi.org/10.1111/1346-8138.12869