# Huriez Syndrome and SCC Risk: A Narrative Review Highlighting Surgical Challenges and Oncologic Considerations

## TL;DR

Huriez syndrome is a rare skin condition linked to aggressive skin cancer, requiring early diagnosis and multidisciplinary care to manage surgical and oncologic challenges.

## Contribution

This review highlights the surgical and oncologic challenges in Huriez syndrome and emphasizes the need for early detection and multidisciplinary care.

## Key findings

- Most patients required multiple surgeries due to recurrent or bilateral disease.
- Amputations were common for advanced cases, with successful flap reconstructions remaining disease-free.
- Local recurrence was reported in 41.6% of cases, underscoring the need for vigilant monitoring.

## Abstract

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.