Dataset Information

Pathological characterization of pachydermia in pachydermoperiostosis.

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.

SUBMITTER: Tanese K

PROVIDER: S-EPMC5029778 | biostudies-literature | 2015 Jul

REPOSITORIES: biostudies-literature

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Pathological characterization of pachydermia in pachydermoperiostosis.

Tanese Keiji K Niizeki Hironori H Seki Atsuhito A Otsuka Atsushi A Kabashima Kenji K Kosaki Keisuke K Kuwahara Masamitsu M Miyakawa Shun-Ichi S Miyasaka Mikiko M Matsuoka Kentaro K Okuyama Torayuki T Shiohama Aiko A Sasaki Takashi T Kudoh Jun J Amagai Masayuki M Ishiko Akira A

The Journal of dermatology 20150511 7

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, w ...[more]

PMID: 25964088

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Dataset Information

Pathological characterization of pachydermia in pachydermoperiostosis.

Publications

Pathological characterization of pachydermia in pachydermoperiostosis.

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