Pathological characterization of pachydermia in pachydermoperiostosis

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