Pachydermoperiostosis, a unique entity with distinctive clinical features

Abstract

Pachydermoperiostosis, which occurs more frequently in men, is a rare entity with distinctive clinical features and an insidious onset.. We report the case of a 30-year-old man with a one-year history of acropachy, arthralgias, hiperhidrosis, and progressive skin thickening of the face and scalp.The radiological findings were consistent with periostosis and the histopathological analysis from a facial skin biopsy showed a pandermal increase in the thickness and number of collagen bundles.The pathogenesis of PDP is currently unknown, although an increased secretion of prostaglandin E2 (PGE2), which stimulates the overexpression of vascular endothelial growth factor (VEGF), has been suggested as a major factor. No specific treatment exists; however, in most cases, the disease tends to stabilize over time.

Comprehensive Treatment of a Rare Case of Complete Primary Pachydermoperiostosis with Large Facial Keloid Scars: A Case Report and Literature Review

“…No specific treatment exists for PDH. However, in most cases, the PDP tends to stabilize over time [ 14 ]. Plastic surgery can be recommended for patients with complete primary PDH to improve their quality of life.…”