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Keratosis palmoplantaris striata
3 OMIM references -
3 associated genes
100 connected diseases
4 signs/symptoms
Disease Type of connection
Familial isolated arrhythmogenic ventricular dysplasia, biventricular form
Familial isolated arrhythmogenic ventricular dysplasia, left dominant form
Familial isolated arrhythmogenic ventricular dysplasia, right dominant form
Lethal acantholytic epidermolysis bullosa
Annular epidermolytic ichthyosis
Epidermolytic ichthyosis
Epidermolytic palmoplantar keratoderma
Ichthyosis hystrix of Curth-Macklin
Idiopathic pulmonary fibrosis
Skin fragility-woolly hair-palmoplantar keratoderma syndrome
Woolly hair-palmoplantar keratoderma-dilated cardiomyopathy syndrome
Diffuse palmoplantar keratoderma with painful fissures
Focal palmoplantar keratoderma with joint keratoses
Severe dermatitis-multiple allergies-metabolic wasting syndrome
Leber congenital amaurosis
Senior-Loken syndrome
Spinocerebellar ataxia type 12
Estrogen resistance syndrome
Naxos disease
Epidermolysis bullosa simplex due to plakophilin deficiency
Exfoliative ichthyosis
Dedifferentiated liposarcoma
Well-differentiated liposarcoma
Amyotrophic lateral sclerosis
Familial isolated dilated cardiomyopathy
Fanconi anemia
Isolated growth hormone deficiency type IA
Isolated growth hormone deficiency type IB
Isolated growth hormone deficiency type II
Pseudohypoaldosteronism type 2E
Short stature due to growth hormone qualitative anomaly
Behavioral variant of frontotemporal dementia
Progressive non-fluent aphasia
Semantic dementia
Dowling-Degos disease
Epidermolysis bullosa simplex with circinate migratory erythema
Epidermolysis bullosa simplex with mottled pigmentation
Epidermolysis bullosa simplex, Dowling-Meara type
Generalized epidermolysis bullosa simplex, non-Dowling-Meara type
Localized epidermolysis bullosa simplex
Autosomal dominant limb-girdle muscular dystrophy type 1E
Autosomal recessive limb-girdle muscular dystrophy due to desmin deficiency
Desminopathy
Pulverulent cataract
Scapuloperoneal amyotrophy
Familial gastric cancer
Gastric linitis plastica
Congenital high-molecular-weight kininogen deficiency
Keratoderma hereditarium mutilans with ichthyosis
Progressive symmetric erythrokeratodermia
Adult-onset distal myopathy due to VCP mutation
Inclusion body myopathy with Paget disease of bone and frontotemporal dementia
Spastic paraplegia - Paget disease of bone
APC-related attenuated familial adenomatous polyposis
Apolipoprotein A-I deficiency
Common variable immunodeficiency
Congenital analbuminemia
Desmoid tumor
Familial adenomatous polyposis due to 5q22.2 microdeletion
Familial renal amyloidosis due to Apolipoprotein AI variant
Fibronectin glomerulopathy
Gardner syndrome
Primary systemic amyloidosis
Richieri Costa-Pereira syndrome
Turcot syndrome with polyposis
Generalized junctional epidermolysis bullosa, non-Herlitz type
Late-onset junctional epidermolysis bullosa
Localized junctional epidermolysis bullosa, non-Herlitz type
46,XY partial gonadal dysgenesis
Autosomal agammaglobulinemia
Autosomal recessive cerebellar ataxia - epilepsy - intellectual deficit
B-cell chronic lymphocytic leukemia
Burkitt lymphoma
Cabezas syndrome
Congenital bilateral absence of vas deferens
Cystic fibrosis
Frontotemporal dementia with motor neuron disease
Giant cell glioblastoma
Gliosarcoma
Glycogen storage disease due to muscle beta-enolase deficiency
Hereditary chronic pancreatitis
Idiopathic bronchiectasis
Juvenile myelomonocytic leukemia
Male infertility with normal virilization due to meiosis defect
Megalencephaly - polymicrogyria - postaxial polydactyly - hydrocephalus
Noonan syndrome-like disorder with juvenile myelomonocytic leukemia
Opsismodysplasia
Papillary or follicular thyroid carcinoma
Pontocerebellar hypoplasia type 1
Precursor T-cell acute lymphoblastic leukemia
Pyogenic arthritis - pyoderma gangrenosum - acne
Pachyonychia congenita
Autoimmune lymphoproliferative syndrome
Congenital factor XII deficiency
Hereditary angioedema type 3
Autosomal recessive nonsyndromic intellectual deficit
Congenital reticular ichthyosiform erythroderma
Early-onset autosomal dominant Alzheimer disease
Reticular dysgenesis
Superficial epidermolytic ichthyosis
Synonym(s):
- Keratosis palmoplantaris striata et areata
- Keratosis palmoplantaris varians of Wachters
- Striate palmoplantar keratoderma
Classification (Orphanet):
- Rare genetic disease
- Rare skin disease
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -
Epidemiological data:
Class of prevalence: -
Average age onset: -
Average age of death: -
Type of inheritance: autosomal dominant
External references:
3 OMIM references -
No MeSH references
Gene symbol UniProt reference OMIM reference
DSG1 Q02413125670
DSP P15924125647
KRT1 P04264139350
Very frequent
- Autosomal dominant inheritance
- Palmoplantar hyperkeratosis / keratoderma

Frequent
- Hair and scalp anomalies
- Nails anomalies