PDF(Pubmed)
Abstract：
UNASSIGNED: Pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy, is a rare autosomal dominant disease with primary clinical features of pachydermia (thickening of skin) and periostosis (new bone formation). Keloid scar formation is also rather obscure, and some scientists have claimed that keloid scars contain an excessive amount of fibroblasts compared with normal skin as well as a dense mass of irregularly deposited connective tissues.
UNASSIGNED: A 25-year-old man exhibited extensive skin folding on his face, a gyrus-like scalp, depressed nasolabial folds, and keloids. Symptoms began at 18 years of age, progressing insidiously. Additionally, he experienced clubbing of fingers and toes, joint pain, muscle soreness, and hyperhidrosis. Radiographic examinations revealed thickened bone and cystic regions. Diagnosed with complete primary PDP and facial keloid scars, he underwent skin dermabrasion, biopsies, and a comprehensive treatment involving, botulinum toxin injections, 5-fluorouracil, and a carbon dioxide lattice laser.
UNASSIGNED: PDP presents challenges due to its unclear etiology but stabilizes over time in most cases. Comprehensive treatment strategies, including dermabrasion and a combination of intralesional therapies, are effective in managing keloids in PDP patients. This case contributes to the understanding of managing rare diseases and underscores the importance of personalized approaches to improve therapeutic outcomes in patients with complete primary PDP and concurrent keloids.
UNASSIGNED: A 25-year-old man exhibited extensive skin folding on his face, a gyrus-like scalp, depressed nasolabial folds, and keloids. Symptoms began at 18 years of age, progressing insidiously. Additionally, he experienced clubbing of fingers and toes, joint pain, muscle soreness, and hyperhidrosis. Radiographic examinations revealed thickened bone and cystic regions. Diagnosed with complete primary PDP and facial keloid scars, he underwent skin dermabrasion, biopsies, and a comprehensive treatment involving, botulinum toxin injections, 5-fluorouracil, and a carbon dioxide lattice laser.
UNASSIGNED: PDP presents challenges due to its unclear etiology but stabilizes over time in most cases. Comprehensive treatment strategies, including dermabrasion and a combination of intralesional therapies, are effective in managing keloids in PDP patients. This case contributes to the understanding of managing rare diseases and underscores the importance of personalized approaches to improve therapeutic outcomes in patients with complete primary PDP and concurrent keloids.
摘要：
■厚皮层骨病（PDP），或者原发性肥大性骨关节病,是一种罕见的常染色体显性疾病，其主要临床特征为厚皮症（皮肤增厚）和骨膜增生（新骨形成）。瘢痕瘢痕形成也相当模糊，一些科学家声称，与正常皮肤相比，瘢痕疙瘩疤痕含有过量的成纤维细胞，以及大量不规则沉积的结缔组织。
■一名25岁的男子脸上表现出广泛的皮肤折叠，像回一样的头皮，凹陷的鼻唇沟，还有瘢痕疙瘩.症状始于18岁，阴险地进步。此外,他经历了手指和脚趾的撞击，关节痛,肌肉酸痛,和多汗症。影像学检查显示骨骼增厚和囊性区域。诊断为完整的原发性PDP和面部瘢痕疙瘩疤痕，他接受了皮肤磨皮术,活检,和综合治疗，肉毒杆菌毒素注射,5-氟尿嘧啶,和二氧化碳晶格激光器。
■PDP由于其病因不清楚而面临挑战，但在大多数情况下随着时间的推移而稳定。综合治疗策略,包括磨皮术和病灶内治疗的组合，有效地管理PDP患者的瘢痕疙瘩。此病例有助于了解管理罕见疾病，并强调个性化方法的重要性，以改善患有完全原发性PDP和并发瘢痕疙瘩的患者的治疗结果。
■一名25岁的男子脸上表现出广泛的皮肤折叠，像回一样的头皮，凹陷的鼻唇沟，还有瘢痕疙瘩.症状始于18岁，阴险地进步。此外,他经历了手指和脚趾的撞击，关节痛,肌肉酸痛,和多汗症。影像学检查显示骨骼增厚和囊性区域。诊断为完整的原发性PDP和面部瘢痕疙瘩疤痕，他接受了皮肤磨皮术,活检,和综合治疗，肉毒杆菌毒素注射,5-氟尿嘧啶,和二氧化碳晶格激光器。
■PDP由于其病因不清楚而面临挑战，但在大多数情况下随着时间的推移而稳定。综合治疗策略,包括磨皮术和病灶内治疗的组合，有效地管理PDP患者的瘢痕疙瘩。此病例有助于了解管理罕见疾病，并强调个性化方法的重要性，以改善患有完全原发性PDP和并发瘢痕疙瘩的患者的治疗结果。
