Patrignani A, Ribuffo D, Greco A, Lo Torto F, Pagnotta A
Department of Plastic Reconstructive and Aesthetic Surgery, Policlinico Umberto I, Sapienza University of Rome, Viale del Policlinico155, 00161 Rome, Italy.
Hand and Microsurgery Unit, Jewish Hospital, Via Fulda 14, Rome 00148, Italy.
JPRAS Open. 2024 Nov 28;43:180-186. doi: 10.1016/j.jpra.2024.11.014. eCollection 2025 Mar.
Huriez syndrome is a rare de rmatological condition characterized by severe sclerotic and atrophic changes in the extremities (hands and feet) and an increased tendency to develop squamous cell carcinomas, with no established gold standard for the surgical treatment of these patients, who are difficult to manage due to the inability to perform reconstructions using local flaps.
We report the case of a patient with severe Huriez syndrome who had developed SCC in both the hands and foot over time. After multiple surgeries at other centers, all resulting in recurrences, we planned wide excisions followed by reconstruction using distant flaps. The postoperative course for both reconstructive procedures was complication-free. Follow-up revealed not only an excellent reconstructive outcome but also highlighted the untapped potential of the flap in managing this condition: thanks to its independent vascularization, the flap remained unaffected by the surrounding diseased skin and significantly improved the scaly, atrophic appearance of the affected areas.
Huriez syndrome is a dermatological condition characterized by a predisposition to developing squamous cell carcinomas. In these patients, it is essential to plan wide excisions that ensure oncological radicality. The reconstruction must be carefully planned, and microvascular flaps can be prioritized.
The radial forearm flap has proven effective for reconstruction in individuals with SCC associated with Huriez syndrome. The skin of the flap not only remained unaffected by the surrounding pathology but also induced a previously undescribed benefit on the diseased skin. This oncological-reconstructive approach could become the gold standard in treating these patients.
Huriez综合征是一种罕见的皮肤病，其特征为四肢（手和脚）出现严重的硬化和萎缩性改变，且患鳞状细胞癌的倾向增加，对于这些患者的手术治疗尚无既定的金标准，由于无法使用局部皮瓣进行重建，这些患者难以处理。
我们报告一例患有严重Huriez综合征的患者，随着时间的推移，其双手和双脚均发生了鳞状细胞癌。在其他中心进行多次手术后，均导致复发，我们计划进行广泛切除，然后使用远位皮瓣进行重建。两种重建手术的术后过程均无并发症。随访不仅显示出出色的重建效果，还突出了该皮瓣在处理这种疾病方面尚未开发的潜力：由于其独立的血管供应，该皮瓣不受周围病变皮肤的影响，并显著改善了受影响区域的鳞状、萎缩外观。
Huriez综合征是一种皮肤病，其特征是易患鳞状细胞癌。对于这些患者，计划进行确保肿瘤根治性的广泛切除至关重要。重建必须精心规划，微血管皮瓣可作为首选。
桡侧前臂皮瓣已被证明对与Huriez综合征相关的鳞状细胞癌患者的重建有效。该皮瓣的皮肤不仅不受周围病变的影响，而且对病变皮肤产生了以前未描述过的益处。这种肿瘤重建方法可能成为治疗这些患者的金标准。
