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Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal.
- Source :
-
The British journal of dermatology [Br J Dermatol] 1995 Sep; Vol. 133 (3), pp. 409-16. - Publication Year :
- 1995
-
Abstract
- Keratoderma with scleroatrophy of the extremities, also referred to as Huriez syndrome, is a rare, autosomal dominant condition, first described in 42 of 132 members of two families from northern France. The term sclerotylosis was proposed because of the pseudosclerodermatous appearance of the hands and digits. The distinctive feature of this syndrome is the risk of the development of squamous cell carcinoma on affected skin. Since the initial description of this disease, three other families, and possibly a fourth, have been reported. In the present study, we reassessed the clinical, pathological and genetic data in 114 members of one of the two original families, of whom 27 were affected by this syndrome.
- Subjects :
- Acitretin therapeutic use
Adult
Carcinoma, Squamous Cell complications
DNA Repair
Drug Therapy, Combination
Etretinate therapeutic use
Female
Genetic Linkage
Humans
Keratoderma, Palmoplantar complications
Keratoderma, Palmoplantar genetics
Keratolytic Agents therapeutic use
Male
Middle Aged
Pedigree
Skin Neoplasms complications
Keratoderma, Palmoplantar pathology
Subjects
Details
- Language :
- English
- ISSN :
- 0007-0963
- Volume :
- 133
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The British journal of dermatology
- Publication Type :
- Academic Journal
- Accession number :
- 8546996
- Full Text :
- https://doi.org/10.1111/j.1365-2133.1995.tb02669.x