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. 2018 Apr;4(2):82-85.
doi: 10.1159/000479036. Epub 2017 Aug 12.

A Rare Syndrome Resembling Scleroderma: Huriez Syndrome

Nil Su Çelik  1 , Şirin Yaşar  1 , Sema Aytekin  1 , Pembegül Güneş  2
Affiliations

A Rare Syndrome Resembling Scleroderma: Huriez Syndrome

Nil Su Çelik et al. Skin Appendage Disord. 2018 Apr.

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.

Keywords: Autosomal dominant inheritance; Keratoderma; Nail disorder; Scleroderma-like findings; Sclerosis; Squamous cell carcinoma.

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Figures

Fig. 1
Fig. 1
The patient presented with acrosclerosis, hardening of fingers, and hypoplastic nails.
Fig. 2
Fig. 2
Nail dystrophy and distal thinning. The “prayer sign” was observed.
Fig. 3
Fig. 3
Plantar thick keratoderma.
Fig. 4
Fig. 4
Hypergranulosis, irregular acanthosis, and mild papillomatosis. HE. ×200.
See this image and copyright information in PMC

References

    1. Huriez C, Agache P, Bombart M, Souilliart F. Spinocellular epitheliomas in congenital cutaneous atrophy in 2 families with high cancer morbidity. Bull Soc Fr Dermatol Syphiligr. 1963;70:24–28. - PubMed
    1. Sekar SC, Srinivas CR. Huriez syndrome. Indian J Dermatol Venereol Leprol. 2008;74:409–410. - PubMed
    1. Al Aboud K, Khachemoune A. Claude Huriez and his syndrome. Skinmed. 2011;9:313–314. - PubMed
    1. Watanabe E, Takai T, Ichihashi M, Ueda M. A nonfamilial Japanese case of Huriez syndrome: P53 expression in squamous cell carcinoma. Dermatology. 2003;207:82–84. - PubMed
    1. Vernole P, Terrinoni A, Didona B, De Laurenzi V, Rossi P, Melino G, et al. An SRY-negative XX male with Huriez syndrome. Clin Genet. 2000;57:61–66. - PubMed