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Review
. 2025 Jul 23;14(15):5214.
doi: 10.3390/jcm14155214.

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

Alessia Pagnotta  1 , Luca Patanè  2 , Carmine Zoccali  3 , Francesco Saverio Loria  2 , Federico Lo Torto  2 , Diego Ribuffo  2
Affiliations
Review

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

Alessia Pagnotta et al. J Clin Med. .

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.

Keywords: Huriez syndrome; genodermatoses; rare diseases; skin cancer; squamous cell carcinoma.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
A patient with Huriez syndrome and a cutaneous SCC of the second finger.
Figure 2
Figure 2
PRISMA flow diagram. (1) Not in English, letter, book section, experimental, animal studies.
Figure 3
Figure 3
The same patient as in Figure 1, following the second finger amputation. The patient healed well with optimal functional recovery.
See this image and copyright information in PMC

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