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Ichthyosis vulgaris: Who gets and causes


Who gets ichthyosis vulgaris?

To get ichthyosis vulgaris, one of the following must happen. The person can:

  • Inherit genes for ichthyosis from one or both parents

  • Develop a gene mutation for ichthyosis while in the womb

  • Get a disease or take a medicine that can trigger ichthyosis

When caused by genes, the disease appears during childhood, usually between 3 months and 5 years of age. This type of ichthyosis vulgaris is called inherited ichthyosis vulgaris. It often runs in families.

The other type of ichthyosis vulgaris is very rare. Called acquired ichthyosis vulgaris, this type usually affects adults only. It appears when it is triggered by a disease or medicine.

People of all races get ichthyosis vulgaris. About the same number of males and females develop this disease.

What causes ichthyosis vulgaris?

Inherited ichthyosis vulgaris is caused by genes. The person either inherits the genes for this disease from one or both parents or genes changes while the baby is forming inside the womb. As a result, the skin has less filaggrin than it needs. The body needs filaggrin to create a healthy outermost layer of skin.

Without enough filaggrin, the body cannot shed skin cells as it should. The older skin cells build up on the skin’s surface, causing the scale and thickened skin.

Acquired ichthyosis vulgaris has a different cause. A disease usually triggers it. Diseases that can trigger it include long-term kidney failure, different types of cancer (especially lymphoma), sarcoidosis, and infections such as leprosy and HIV.

Acquired ichthyosis vulgaris can show up before the person has signs of the triggering disease. If this happens, the person should have a complete physical. It’s important to find the cause.

In very rare cases, a medicine or vitamin can trigger acquired ichthyosis vulgaris. Ones that can do this, include:

  • Cimetidine (an antacid and antihistamine used to treat ulcers and acid reflux)

  • Clofazimine (a drug used to treat leprosy)

  • Nicotinic acid, a B vitamin


References
Brown SJ, McLean WH. “One remarkable molecule: filaggrin.” J Invest Dermatol. 2012 Mar;132(3 Pt 2):751-62.

James WD, Berger TG, et al. “Genodermatoses and congenital anomalies.” In: James WD, Berger TG, et al. Andrews' Diseases of the Skin, Clinical Dermatology (tenth edition), Elsevier, Canada, 2006:560-1.

Richard G and Ringpfeil F. “Ichthyoses, erythrokeratodermas, and related disorders.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:743-9.

Schwartz RA. “Hereditary and acquired ichthyosis vulgaris.” In: Elston DM, (chief editor) Medscape. Last updated June 22, 2016.

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