| Title: |
Huriez syndrome and SCC risk: a narrative review highlighting surgical challenges and oncologic considerations |
| Authors: |
Pagnotta A.; Patane L.; Zoccali C.; Loria F. S.; Lo Torto F.; Ribuffo D. |
| Contributors: |
Pagnotta, A.; Patane, L.; Zoccali, C.; Loria, F. S.; Lo Torto, F.; Ribuffo, D. |
| Publisher Information: |
MDPI; Basel |
| Publication Year: |
2025 |
| Collection: |
Sapienza Università di Roma: CINECA IRIS |
| Subject Terms: |
huriez syndrome; genodermatose; rare disease; skin cancer; squamous cell carcinoma |
| Description: |
Background: Huriez syndrome is a rare hereditary skin disorder marked by early-onset sclerodactyly, hyperkeratosis of the palms and soles, and nail dysplasia. A key concern is the early and aggressive development of cutaneous squamous cell carcinoma (SCC), typically affecting the dorsal aspects of the hands. Methods: This narrative review summarizes clinical features, genetic aspects, and oncologic implications of Huriez syndrome. A systematic search was conducted in PubMed and Scopus, including English-language articles published up to May 2025. Relevant case reports and small case series were analyzed. Results: Seven patients (58.3%) underwent multiple surgeries due to recurrent or bilateral disease. Six patients (50%) required amputations, including finger, hand, and arm amputations, with no foot amputations reported. Reconstruction after oncological resection was performed in six patients (50%) using skin grafts (3), pedicled flaps (2), or free flaps (1). Amputation was mainly for advanced disease, with radial forearm flaps used for reconstruction. All flaps remained disease-free. Five cases (41.6%) had a history of local recurrence. Conclusions: The early diagnosis of Huriez syndrome is crucial to enable the surveillance and timely treatment of SCC. A multidisciplinary team including dermatologists, oncologists, plastic surgeons, and geneticists is recommended. Further research is needed to clarify genetic mechanisms and develop early detection strategies to improve outcomes. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
info:eu-repo/semantics/altIdentifier/pmid/40806836; info:eu-repo/semantics/altIdentifier/wos/WOS:001548812600001; volume:14; issue:15; firstpage:1; lastpage:10; numberofpages:10; journal:JOURNAL OF CLINICAL MEDICINE; https://hdl.handle.net/11573/1751355 |
| DOI: |
10.3390/jcm14155214 |
| Availability: |
https://hdl.handle.net/11573/1751355; https://doi.org/10.3390/jcm14155214 |
| Rights: |
info:eu-repo/semantics/openAccess ; license:Creative commons ; license uri:http://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.A08EA51E |
| Database: |
BASE |