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NORD is very grateful to the medical advisors of the Foundation for Ichthyosis & Related Skin Types for assistance in the preparation of this report.
Synonyms of Ichthyosis
- Disorders of Cornification
- Chanarin-Dorfman syndrome (neutral lipid storage disease)
- CHILD syndrome (unilateral hemidysplasia)
- congenital ichthyosiform erythroderma (CIE)
- Conradi-Hunermann syndrome (X-linked dominant chondrodysplasia punctata)
- Darier disease
- epidermal nevi (ichthyosis hystrix, linear epidermal nevus)
- epidermolytic hyperkeratosis (EHK)
- erythrokeratodermia variabilis (EKV)
- Giroux-Barbeau syndrome
- Hailey-Hailey disease (benign familial pemphigus)
- harlequin ichthyosis (harlequin fetus)
- ichthyosis hystrix Curth-Macklin type
- ichthyosis vulgaris (ichthyosis simplex)
- keratosis follicularis spinulosa decalvans
- KID syndrome (keratitis, ichthyosis, deafness)
- lamellar ichthyosis
- multiple sulfatase deficiency
- Netherton syndrome (ichthyosis linearis circumflexa)
- pachyonychia congenita
- palmoplantar keratodermas (PPK)
- peeling skin syndrome
- pityriasis rubra pilaris (PRP)
- Refsum's disease (phytanic acid storage disease)
- Rud's syndrome
- Sjogren-Larsson syndrome
- Tay's syndrome (trichothiodystrophy, IBIDS syndrome)
- X-linked ichthyosis
Ichthyosis is a general term for a family of rare genetic skin diseases characterized by dry, thickened, scaling skin. The various forms are distinguished from one another by: 1) extent of the scaling and how widely and where the scaling is scattered over the body; 2) the presence or absence and intensity of reddening of the skin (erythroderma); 3) the mode of inheritance; and 4) the character of associated abnormalities.
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