Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal.
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.
Delaporte
E
E
Service de Dermatologie A, Hôpital Claude Huriez, CHRU, Lille, France.
N'guyen-Mailfer
C
C
Janin
A
A
Savary
J B
JB
Vasseur
F
F
Feingold
N
N
Piette
F
F
Bergoend
H
H
D017255:Acitretin / Q000627:therapeutic use; D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000150:complications; D004260:DNA Repair; D004359:Drug Therapy, Combination; D005050:Etretinate / Q000627:therapeutic use; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000150:complications / Q000235:genetics / Q000473:pathology*; D007641:Keratolytic Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012878:Skin Neoplasms / Q000150:complications
D007641:Keratolytic Agents; D005050:Etretinate; D017255:Acitretin