page_title: List of skin conditions
section_title: Genodermatoses
hierarchical_section_title: List of skin conditions / Genodermatoses
caption_attribution_description: English: Boy with polygonal scales since birth. Brother, maternal uncle, and maternal grandfather affected.
context_page_description: Many conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square metres, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles, and hair-bearing skin. Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm that forms the dermis and subcutaneous tissues.
  The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. Nourishment is provided to these layers by diffusion from the dermis, since the epidermis is without direct blood supply.
context_section_description: Genodermatoses are inherited genetic skin conditions often grouped into three categories: chromosomal, single gene, and polygenetic.
  18q deletion syndrome
  Acrodermatitis enteropathica
  Acrogeria (Gottron syndrome)
  Acrokeratosis verruciformis (acrokeratosis verruciformis of Hopf)
  Adams–Oliver syndrome
  Adducted thumbs syndrome
  Albright's hereditary osteodystrophy
  Angelman syndrome
  Apert syndrome (acrocephalosyndactyly)
  Arthrogryposis–renal dysfunction–cholestasis syndrome
  Ataxia telangiectasia (Louis–Bar syndrome)
  Atrichia with papular lesions (papular atrichia)
  Atrophodermia vermiculata (acne vermoulante, acne vermoulanti, atrophoderma reticulata symmetrica faciei, atrophoderma reticulatum, atrophoderma vermiculata, atrophoderma vermiculatum, atrophodermia reticulata symmetrica faciei, atrophodermia ulerythematosa, atrophodermie vermiculée des joues avec kératoses folliculaires, folliculitis ulerythema reticulata, folliculitis ulerythematous reticulata, folliculitis ulerythemosa, honeycomb atrophy, ulerythema acneforme, ulerythema acneiforme)
  Autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy syndrome
  Bart syndrome
  Bazex–Dupré–Christol syndrome (Bazex syndrome, follicular atrophoderma and basal cell cacinomas)
  Beare–Stevenson cutis gyrata syndrome
  Bloom syndrome (Bloom–Torre–Machacek syndrome)
  Blue rubber bleb nevus syndrome
  Brittle hair–intellectual impairment–decreased fertility–short stature syndrome
  Cantú syndrome
  Cardio-facio-cutaneous syndrome (cardiofaciocutaneous syndrome)
  Cartilage–hair hypoplasia (McKusick type metaphyseal chondrodysplasia)
  Cerebral dysgenesis–neuropathy–ichthyosis–keratoderma syndrome
  Childhood tumor syndrome
  Chondrodysplasia punctata
  Cicatricial junctional epidermolysis bullosa
  Craniosynostosis–anal anomalies–porokeratosis syndrome
  Cockayne syndrome
  Colobomas of the eye–heart defects–ichthyosiform dermatosis–mental retardation–ear defects syndrome (CHIME syndrome, Zunich neuroectodermal syndrome, Zunich–Kaye syndrome)
  Congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome (CHILD syndrome)
  Conradi–Hünermann syndrome (Conradi–Hünermann–Happle syndrome, Happle syndrome, X-linked dominant chondrodysplasia punctata)
  Costello syndrome
  Cronkhite–Canada syndrome
  Crouzon syndrome
  Cutis verticis gyrata
  Darier's disease (Darier–White disease, dyskeratosis follicularis, keratosis follicularis) 
  DeSanctis–Cacchione syndrome
  Disseminated superficial actinic porokeratosis
  Disseminated superficial porokeratosis
  Dolichol kinase deficiency
  Dominant dystrophic epidermolysis bullosa
  Dyskeratosis congenita (Zinsser–Cole–Engman syndrome)
  Dystrophic epidermolysis bullosa
  Ectodermal dysplasia
  Ectodermal dysplasia with corkscrew hairs
  Ectrodactyly–ectodermal dysplasia–cleft syndrome (EEC syndrome, split hand–split foot–ectodermal dysplasia–cleft syndrome)
  Epidermolysis bullosa herpetiformis (Dowling–Meara epidermolysis bullosa simplex)
  Epidermolysis bullosa simplex
  Epidermolysis bullosa simplex of Ogna
  Epidermolysis bullosa simplex with mottled pigmentation
  Epidermolysis bullosa simplex with muscular dystrophy
  Epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma, bullous ichthyosiform erythroderma)
  Erythrokeratodermia with ataxia (Giroux–Barbeau syndrome)
  Familial benign chronic pemphigus (familial benign pemphigus, Hailey–Hailey disease)
  Fanconi syndrome (familial pancytopenia, familial panmyelophthisis)
  Fibrodysplasia ossificans progressiva
  Focal dermal hypoplasia (Goltz syndrome)
  Follicular atrophoderma
  Franceschetti–Klein syndrome (mandibulofacial dysostosis)
  Gardner's syndrome (familial colorectal polyposis)
  Gastrocutaneous syndrome
  Generalized atrophic benign epidermolysis bullosa
  Generalized epidermolysis bullosa simplex (Koebner variant of generalized epidermolysis bullosa simplex)
  Generalized trichoepithelioma
  Giant axonal neuropathy with curly hair
  Gingival fibromatosis with hypertrichosis
  Haber syndrome
  Hallerman–Streiff syndrome
  Harlequin-type ichthyosis (harlequin baby, harlequin fetus, harlequin ichthyosis, ichthyosis congenita, ichthyosis con
caption: X-linked ichthyosis
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