Pathological characterization of pachydermia in pachydermoperiostosis
| Autor: | Masamitsu Kuwahara, Keisuke Kosaki, Atsushi Otsuka, Jun Kudoh, Akira Ishiko, Atsuhito Seki, Masayuki Amagai, Aiko Shiohama, Hironori Niizeki, Shun Ichi Miyakawa, Keiji Tanese, Takashi Sasaki, Kentaro Matsuoka, Kenji Kabashima, Torayuki Okuyama, Mikiko Miyasaka |
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| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Osteoarthropathy Primary Hypertrophic Concise Communications Sebaceous hyperplasia Dermatology sebaceous hyperplasia Skin Diseases Young Adult Fibrosis Biopsy medicine Humans mucin deposition Forehead dermal edema pachydermoperiostosis biology medicine.diagnostic_test business.industry Concise Communication fibrosis elastic fiber degeneration General Medicine Hyperplasia Middle Aged medicine.disease biology.organism_classification medicine.anatomical_structure Pachydermia Skin biopsy Cutis verticis gyrata business Elastic fiber Facial Dermatoses |
| Zdroj: | The Journal of Dermatology |
| ISSN: | 1346-8138 0385-2407 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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