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Ichthyosis vulgaris (also known as "autosomal dominant ichthyosis" [1] and "Ichthyosis simplex" [1]) is a skin disorder causing dry, scaly skin. It is the most common form, and one of the mildest forms, of ichthyosis , [ 2 ] [ 3 ] : 486 affecting around 1 in 250 people. [ 4 ]
Treatments may include moisturizing cream, antibiotics, etretinate or retinoids. [3] [5] Around half of those affected die within the first few months; [7] however, retinoid treatment can increase chances of survival. [9] [8] Children who survive the first year of life often have long-term problems such as red skin, joint contractures and ...
Ichthyosis vulgaris (autosomal dominant ichthyosis, ichthyosis simplex) Ichthyosis with confetti; Neonatal ichthyosis–sclerosing cholangitis syndrome (ichthyosis–sclerosing cholangitis syndrome, NISCH syndrome) Incontinentia pigmenti achromians (hypomelanosis of Ito) Immune dysfunction–polyendocrinopathy–enteropathy–X-linked syndrome
Ichthyosis (also named fish scale disease) [1] is a family of genetic skin disorders characterized by dry, thickened, scaly skin. [2] The more than 20 types of ichthyosis range in severity of symptoms, outward appearance, underlying genetic cause and mode of inheritance (e.g., dominant, recessive, autosomal or X-linked). [3]
Sjögren–Larsson syndrome is a rare autosomal recessive form of ichthyosis with neurological symptoms. [ 1 ] : 485 [ 2 ] : 564 [ 3 ] It can be identified by a triad of medical disorders. The first is ichthyosis , which is a buildup of skin to form a scale-like covering that causes dry skin and other problems.
Survival rates based on the severity of the case have varied from 10 months to 25 years with supportive treatment. The oldest person in the united states with Harlequin Ichthyosis is 23 year old Stephanie Turner. [10] She was also the first person to have a child with this condition. She has two children and neither have the condition.
Intravenous immunoglobulin has become established as the treatment of choice in Netherton syndrome. [6] This therapy reduces infection; enables improvement and even resolution of the skin and hair abnormalities, and dramatically improves quality of life of the patients; although exactly how it achieves this is not known.
Neonatal ichthyosis–sclerosing cholangitis syndrome (also known as "NISCH syndrome" [1] and "ichthyosis–sclerosing cholangitis syndrome" [1]) is a cutaneous condition which is characterized by hypotrichosis of the scalp, alopecia, ichthyosis and sclerosing cholangitis. [2]
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