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

Area of Science:

• Dermatology
• Oncology
• Genetics

Background:

• Huriez syndrome is a rare genetic skin disorder characterized by sclerodactyly, palmoplantar hyperkeratosis, and nail dysplasia.
• A significant concern is the early and aggressive development of cutaneous squamous cell carcinoma (SCC), primarily on the hands.

Purpose of the Study:

• To review the clinical features, genetic basis, and oncologic implications of Huriez syndrome.
• To highlight the importance of early diagnosis and management of associated SCC.

Main Methods:

• A narrative review of English-language literature from PubMed and Scopus up to May 2025.
• Analysis of case reports and small case series focusing on Huriez syndrome and SCC.

Main Results:

• Multiple surgeries and amputations (finger, hand, arm) were required in 50-58.3% of patients due to recurrent or advanced disease.
• Reconstruction involved skin grafts or flaps, with radial forearm flaps showing disease-free success.
• Local recurrence of SCC was noted in 41.6% of cases.

Conclusions:

• Early diagnosis of Huriez syndrome is critical for SCC surveillance and timely treatment.
• A multidisciplinary team approach involving dermatology, oncology, plastic surgery, and genetics is recommended.
• Further research is needed to elucidate genetic mechanisms and improve early detection strategies.