A Rare Syndrome Resembling Scleroderma: Huriez Syndrome

Skin Appendage Disorders
Nil Su ÇelikPembegül Güneş

Abstract

Huriez syndrome, also referred to as "sclerotylosis," is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nails. The development of aggressive squamous cell carcinoma (SCC) arising in the scleroatrophic area is also a distinctive feature of the syndrome. Early diagnosis is important due to the early onset, mostly in the third to fourth decades of life, and aggressive progress of SCC, which occurs in around 15% of affected individuals. Our patient had palmoplantar keratoderma, scleroatrophy of the hands, and hypoplastic nails. Her mother and father had a second-degree blood relation. Two of her siblings had similar complaints and findings. She showed no sign of actinic keratosis or SCC, and was called for regular follow-ups. With this case, we want to emphasize that Huriez syndrome is a rare genodermatosis, mimicking scleroderma-like acrosclerosis, and early diagnosis is critical for recognizing and preventing the development of SCC.

References

Oct 1, 1995·British Journal of Cancer·F LeviC La Vecchia
Mar 25, 2000·Clinical Genetics·P VernoleP Grimaldi
Jul 2, 2003·Dermatology : International Journal for Clinical and Investigative Dermatology·Eri WatanabeMasato Ueda
Sep 18, 2008·Indian Journal of Dermatology, Venereology and Leprology·Shanmuga C Sekar, C R Srinivas

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Citations

Aug 13, 2021·Frontiers in Immunology·Benjamin Klein, Claudia Günther

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