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Term:
Poikiloderma, Hereditary Sclerosing (DOID:9005023)
Annotations: Rat: (0) Mouse: (0) Human: (1) Chinchilla: (0) Bonobo: (1) Dog: (1) Squirrel: (0)
Parent Terms Term With Siblings Child Terms
Sclerosis +     
Skin Abnormalities +     
Absence of Fingerprints  
acrodermatitis +   
Actinic Prurigo  
Albinism +   
Anetoderma +   
Annular Erythema 
arterial tortuosity syndrome  
atopic dermatitis +   
Atrophia Maculosa Varioliformis Cutis, Familial 
AUTOINFLAMMATION WITH ARTHRITIS AND DYSKERATOSIS  
autosomal recessive congenital ichthyosis +   
Barber-Say syndrome  
Basaloid Follicular Hamartoma Syndrome, Generalized, Autosomal Dominant 
Beare-Stevenson cutis gyrata syndrome  
Blepharophimosis Syndrome Type 1  
Blepharophimosis Syndrome Type 2  
Blepharophimosis with Ptosis, Syndactyly, and Short Stature 
blepharophimosis, ptosis, and epicanthus inversus syndrome  
Bloch-Sulzberger syndrome +   
Book Syndrome 
bullous congenital ichthyosiform erythroderma  
Buschke-Ollendorff Syndrome  
C1q Deficiency  
Carney complex +   
cocoon syndrome  
Collagenosis, Familial Reactive Perforating 
Congenital Erythroderma with Palmoplantar Keratoderma, Hypotrichosis, and Hyper-IgE  
Cutaneous Bullous Amyloidosis 
Cutaneous Hemangiomatosis with Associated Features 
cutaneous porphyria  
cutis laxa +   
Dermal Ridges, Nelson Syndrome 
Dermal Ridges, Patternless 
Dermoodontodysplasia 
Diffuse Mesangial Sclerosis  
Diffuse Renal Mesangial Sclerosis, with Ocular Abnormalities 
Dowling-Degos disease +   
dyschromatosis universalis hereditaria +   
dyskeratosis congenita +   
Dyskeratosis, Hereditary Benign Intraepithelial 
ectodermal dysplasia +   
Ehlers-Danlos syndrome +   
epidermolysis bullosa +   
Epithelial Squamous Dysplasia, Keratinizing Desquamative, of Urinary Tract 
Erosive Arthropathy 
erythrokeratodermia variabilis +   
Erythrokeratodermia with Ataxia  
Exfoliative Ichthyosis, Autosomal Recessive, Ichthyosis Bullosa of Siemens-like  
Familial Dyskeratotic Comedones 
Familial Popliteal Pterygium Syndrome 
Follicular Atrophoderma, Perioral Pigmented, with Milia and Epidermoid Cysts 
Gerodermia Osteodysplastica  
Hailey-Hailey disease  
Hairy Palms and Soles 
Hepatic Porphyrias +   
Hereditary Autoinflammatory Diseases +   
hereditary mucosal leukokeratosis  
Histiocytic Dermatoarthritis 
Hyaluronan Metabolism, Defect in 
Hyperkeratotic Cutaneous Capillary-Venous Malformations Associated with Cerebral Capillary Malformations  
Hypohidrosis with Abnormal Palmar Dermal Ridges 
ichthyosis +   
ichthyosis vulgaris +   
Ichthyosis-Mental Retardation Syndrome with Large Keratohyalin Granules in the Skin 
Infantile Cataract, Skin Abnormalities, Glutamate Excess, and Impaired Intellectual Development  
Isolated Osteopoikilosis 
Juvenile Spring Eruption of Ears 
Keratolytic Winter Erythema  
keratosis follicularis +   
Keratosis Follicularis Spinulosa Decalvans, X-Linked  
Keratosis Linearis with Ichthyosis Congenita and Sclerosing Keratoderma  
lethal restrictive dermopathy  
Leukomelanoderma, Infantilism, Mental Retardation, Hypodontia, Hypotrichosis 
LINEAR SKIN DEFECTS WITH MULTIPLE CONGENITAL ANOMALIES 2  
LINEAR SKIN DEFECTS WITH MULTIPLE CONGENITAL ANOMALIES 3  
lipoid proteinosis  
Mediastinal Fibrosis 
Michelin Tire Baby Syndrome +   
Microphthalmia, Syndromic 7  
monilethrix +   
Muir-Torre syndrome  
Multiple Noduli Cutanei with Urinary Tract Abnormalities 
multiple pterygium syndrome +   
Oculocerebrocutaneous Syndrome 
Oculotrichodysplasia 
orofaciodigital syndrome IX  
Osteosclerosis with Ichthyosis and Premature Ovarian Failure 
PALMOPLANTAR CARCINOMA, MULTIPLE SELF-HEALING  
palmoplantar keratosis +   
Parana Hard Skin Syndrome 
Pedal Onychogryposis with Keratosis Plantaris and Coarse Hair 
peeling skin syndrome +   
Perifolliculitis Capitis Abscedens Et Suffodiens, Familial 
Plasminogen Deficiency, Type I  
poikiloderma with neutropenia  
Poikiloderma, Hereditary Sclerosing +   
porokeratosis +   
Port-Wine Stain +   
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 +   
Pterygium Colli, Isolated 
Reticulate Pigmentary Disorder, with Systemic Manifestations  
Ridges-off-the-end Syndrome 
Rothmund-Thomson syndrome +   
Sclerema Neonatorum 
Sclerosing Bone Dysplasia Mental Retardation 
Sclerosing Lymphocytic Lobulitis 
Sjogren-Larsson syndrome +   
Skin Fragility-Woolly Hair Syndrome  
Skin/Hair/Eye Pigmentation, Variation In, 1  
Skin/Hair/Eye Pigmentation, Variation In, 10  
Skin/Hair/Eye Pigmentation, Variation In, 11  
Skin/Hair/Eye Pigmentation, Variation In, 4  
Skin/Hair/Eye Pigmentation, Variation In, 5  
Skin/Hair/Eye Pigmentation, Variation In, 6  
Skin/Hair/Eye Pigmentation, Variation In, 7  
Skin/Hair/Eye Pigmentation, Variation In, 8  
Skin/Hair/Eye Pigmentation, Variation In, 9  
Stiff Skin Syndrome  
Storm Syndrome 
Systemic Hyalinosis  
Trichothiodystrophy Syndromes +   
Ullrich congenital muscular dystrophy +   
Urban Schosser Spohn Syndrome 
Vascular Hyalinosis 
Vohwinkel Syndrome, Variant Form  
Winter Shortland Temple Syndrome  
X-linked ichthyosis +   
xeroderma pigmentosum +   

Synonyms
Primary IDs: MESH:C562824 ;   RDO:0012375

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