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Pathways
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Ontology Browser
Term:
Parent Terms Term With Siblings Child Terms
keratosis +     
localized scleroderma +     
Skin Neoplasms +     
Abdominal Chemodectomas with Cutaneous Angiolipomas 
acanthoma +   
acquired hyperkeratosis 
acrokeratosis verruciformis  
actinic keratosis +   
Bazex-Dupre-Christol Syndrome 
Becker Nevus Syndrome  
Blue Rubber Bleb Nevus Syndrome  
Brooke-Spiegler syndrome  
Calcifying Epithelial Odontogenic Tumor 
Callosities +  
Cardiomyopathy Hypogonadism Collagenoma Syndrome 
cholesteatoma +   
Curly Hair-Acral Keratoderma-Caries Syndrome 
Davenport Donlan Syndrome 
dermatopathia pigmentosa reticularis  
disabling pansclerotic morphea  
erythrokeratodermia variabilis +   
Familial Cutaneous Collagenoma  
Familial Cutaneous Papillomatosis 
Familial Multiple Trichodiscomas 
Fanconi-like syndrome  
Hamartoma, Precalcaneal Congenital Fibrolipomatous 
Hereditary Leiomyomatosis and Renal Cell Cancer  
hereditary papulotranslucent acrokeratoderma 
Hyperkeratosis Lenticularis Perstans 
ichthyosis +   
Johnston Aarons Schelley Syndrome 
Juvenile Linear Scleroderma 
Juvenile-Onset Scleroderma 
Keratolytic Winter Erythema  
keratosis follicularis +   
large congenital melanocytic nevus  
Lipodermatosclerosis  
Multiple Eruptive Milia 
Palmoplantar Hyperkeratosis with Squamous Cell Carcinoma of Skin and Sex Reversal  
palmoplantar keratoderma and congenital alopecia 2 
palmoplantar keratosis +   
Parakeratosis  
PEELING SKIN WITH LEUKONYCHIA, ACRAL PUNCTATE KERATOSES, CHEILITIS, AND KNUCKLE PADS  
Phacomatosis Pigmentokeratotica 
porokeratosis +   
Progressive Mucinous Histiocytosis 
Reactive Angioendotheliomatosis 
Rombo Syndrome 
Sclerotylosis  
Huriez syndrome (HRZ) is characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nail changes. The development of aggressive squamous cell carcinoma (SCC) in areas of affected skin is a distinctive feature of the syndrome, occurring in approximately 15% of patients. HRZ-associated SCC shows early onset, mostly in the third to fourth decades of life, and early metastasis formation. HRZ is caused by heterozygous mutation in the SMARCAD1 gene on chromosome 4q22. (OMIM)
sebaceous gland neoplasm +   
seborrheic keratosis +   
skin benign neoplasm +   
skin cancer +   
Sweat Gland Neoplasms +   
Trichoepithelioma, Multiple Familial, 2  
Trichoepitheliomas, Multiple Desmoplastic 
Trichofolliculoma 
Trichostasis Spinulosa 
Tufted Angioma  
Urban Schosser Spohn Syndrome  

Synonyms
Exact Synonyms: HRZ ;   Huriez syndrome ;   KERATODERMA WITH SCLEROATROPHY OF THE EXTREMITIES ;   Scleroatrophic and keratotic dermatosis of limbs ;   TYS
Primary IDs: MESH:C537526 ;   OMIM:181600
Alternate IDs: RDO:0003383

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