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Term:
erythrokeratodermia variabilis (DOID:0050467)
Annotations: Rat: (6) Mouse: (6) Human: (6) Chinchilla: (5) Bonobo: (5) Dog: (5) Squirrel: (4)
Parent Terms Term With Siblings Child Terms
Erythema +     
keratosis +     
Absence of Fingerprints  
acquired hyperkeratosis 
acrokeratosis verruciformis  
actinic keratosis +   
Actinic Prurigo  
Albinism +   
Annular Erythema 
arterial tortuosity syndrome  
atopic dermatitis +   
Atrophia Maculosa Varioliformis Cutis, Familial 
AUTOINFLAMMATION WITH ARTHRITIS AND DYSKERATOSIS  
autosomal recessive congenital ichthyosis +   
Basaloid Follicular Hamartoma Syndrome, Generalized, Autosomal Dominant 
Bloch-Sulzberger syndrome +   
bullous congenital ichthyosiform erythroderma  
Buschke-Ollendorff Syndrome  
Callosities +  
cholesteatoma +   
Collagenosis, Familial Reactive Perforating 
Congenital Erythroderma with Palmoplantar Keratoderma, Hypotrichosis, and Hyper-IgE  
Curly Hair-Acral Keratoderma-Caries Syndrome 
Cutaneous Bullous Amyloidosis 
cutaneous porphyria  
cutis laxa +   
Dowling-Degos disease +   
dyschromatosis universalis hereditaria +   
dyskeratosis congenita +   
ectodermal dysplasia +   
Ehlers-Danlos syndrome +   
epidermolysis bullosa +   
Epidermolysis Bullosa Simplex with Migratory Circinate Erythema  
Erythema Chronicum Migrans 
Erythema Induratum 
erythema infectiosum +   
erythema multiforme +   
Erythema Nodosum +   
erythrokeratodermia variabilis +   
An autosomal dominant skin disease characterized by transient and variable noninflammatory ERYTHEMA and hyperkeratosis. It has been associated with mutations in the genes that code for CONNEXINS. Erythrokeratodermia variabilis inherited in an autosomal recessive fashion has also been reported. Affected individuals often develop PALMOPLANTAR KERATODERMA.
Erythrokeratodermia with Ataxia  
Exfoliative Ichthyosis, Autosomal Recessive, Ichthyosis Bullosa of Siemens-like  
Follicular Atrophoderma, Perioral Pigmented, with Milia and Epidermoid Cysts 
Gerodermia Osteodysplastica  
Hailey-Hailey disease  
Hepatic Porphyrias +   
Hereditary Autoinflammatory Diseases +   
hereditary mucosal leukokeratosis  
hereditary papulotranslucent acrokeratoderma 
Histiocytic Dermatoarthritis 
Hooft Disease 
Hyaluronan Metabolism, Defect in 
Hyperkeratosis Lenticularis Perstans 
ichthyosis +   
ichthyosis vulgaris +   
Isolated Osteopoikilosis 
Johnston Aarons Schelley Syndrome 
Juvenile Spring Eruption of Ears 
Keratolytic Winter Erythema  
keratosis follicularis +   
Keratosis Follicularis Spinulosa Decalvans, X-Linked  
Keratosis Linearis with Ichthyosis Congenita and Sclerosing Keratoderma  
Leukomelanoderma, Infantilism, Mental Retardation, Hypodontia, Hypotrichosis 
lipoid proteinosis  
monilethrix +   
Muir-Torre syndrome  
Multiple Eruptive Milia 
Multiple Noduli Cutanei with Urinary Tract Abnormalities 
Oculotrichodysplasia 
orofaciodigital syndrome IX  
palmoplantar keratosis +   
Parakeratosis  
Parana Hard Skin Syndrome 
Pedal Onychogryposis with Keratosis Plantaris and Coarse Hair 
peeling skin syndrome +   
PEELING SKIN WITH LEUKONYCHIA, ACRAL PUNCTATE KERATOSES, CHEILITIS, AND KNUCKLE PADS  
Perifolliculitis Capitis Abscedens Et Suffodiens, Familial 
Plasminogen Deficiency, Type I  
Poikiloderma, Hereditary Sclerosing +   
porokeratosis +   
primary cutaneous amyloidosis  
Primary Localized Cutaneous Amyloidosis, 2  
Primary Localized Cutaneous Amyloidosis, 3  
Progressive Osseous Heteroplasia  
Progressive Vitiligo with Mental Retardation and Urethral Duplication 
Prolidase Deficiency  
pseudoxanthoma elasticum +   
Reticulate Pigmentary Disorder, with Systemic Manifestations  
Rothmund-Thomson syndrome +   
Sclerotylosis 
seborrheic keratosis +   
Sjogren-Larsson syndrome +   
Skin Fragility-Woolly Hair Syndrome  
Stiff Skin Syndrome  
Storm Syndrome 
Sweet Syndrome  
Systemic Hyalinosis  
Trichostasis Spinulosa 
Trichothiodystrophy Syndromes +   
Urban Schosser Spohn Syndrome 
Vohwinkel Syndrome, Variant Form  
X-linked ichthyosis +   
xeroderma pigmentosum +   

Synonyms
Exact Synonyms: EKV ;   EKVP ;   EKVP1 ;   ERYTHROKERATODERMIA VARIABILIS ET PROGRESSIVA ;   ERYTHROKERATODERMIA VARIABILIS ET PROGRESSIVA 1 ;   Erythrokeratodermia Figurata Variabilis ;   Mendes De Costa Syndrome ;   PSEK ;   Progressive Symmetric Erythrokeratodermia ;   erythro et keratodermia variabilis
Primary IDs: MESH:D056266
Alternate IDs: OMIM:133200 ;   RDO:0007739 ;   RDO:0014904
Definition Sources: MESH:D056266, https://en.wikipedia.org/wiki/Erythrokeratodermia_variabilis

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