Ichthyosis vulgaris in Adult
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Synopsis
IV affects approximately 1 in 250-1000 individuals worldwide and is inherited in an autosomal dominant fashion with variable penetrance and expressivity ranging from mild scaling in heterozygotes to severely impaired skin barrier function in homozygotes. There is no known ethnic or sex predilection. There is a strong seasonal and environmental variation in disease severity. The disease is more severe in winter and is exacerbated by low humidity and alkaline skin care products such as soaps and detergents. Hydration, lubrication, and keratolysis are the mainstays of therapy.
IV is primarily caused by loss-of-function mutations in the filaggrin (FLG) gene. Filaggrin is responsible for keratin aggregation during the terminal differentiation of keratinocytes. Altered filaggrin expression results in abnormal cornification of the skin with poor barrier function and increased transepidermal water loss.
The condition typically presents in early childhood and follows a favorable course in which the scaling diminishes in severity by adulthood. Xerotic skin with flaky scales typically develops on the abdomen and extensor surfaces of the extremities with sparing of the flexural surfaces. Hyperkeratosis of the palms and soles accentuate the normal skin creases and in severe cases, can result in painful fissures.
The loss of skin barrier function in IV is associated with an atopic diathesis with over 50% of patients affected by early-onset atopic dermatitis. Keratosis pilaris may be associated.
Codes
ICD10CM:Q80.0 – Ichthyosis vulgaris
SNOMEDCT:
254157005 – Ichthyosis vulgaris
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Differential Diagnosis & Pitfalls
- X-linked ichthyosis in male patients
- Lamellar ichthyosis
- Asteatotic eczema (eczema craquelé)
- Atopic dermatitis
- Xerosis
- Sjögren-Larsson syndrome
- Acquired ichthyosis – Manifests in adulthood and is associated with systemic disease including Hodgkin lymphoma, sarcoidosis, thyroid dysfunction, and human immunodeficiency virus infection.
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Last Reviewed:06/18/2019
Last Updated:01/17/2022
Last Updated:01/17/2022
Patient Information for Ichthyosis vulgaris in Adult
Overview
Ichthyosis vulgaris, also known as common ichthyosis or fish scale disease, is a skin condition resulting in scaly skin, especially on the arms and legs. Its name is derived from the Greek word meaning "fish."Ichthyosis vulgaris can be a trait passed on in families (hereditary), or it may develop later in life as a result of certain exposures (acquired). The hereditary type, also called congenital ichthyosis vulgaris, first appears in early childhood and accounts for more than 95% of cases of ichthyosis vulgaris. The acquired type usually develops in adulthood and results from an internal disease or the use of certain medications.
Who’s At Risk
Ichthyosis vulgaris is found in people of all races and of both sexes. Hereditary ichthyosis vulgaris is fairly common. As many as 1 in 250 children may have hereditary ichthyosis vulgaris. On the other hand, acquired ichthyosis vulgaris is rare and is found almost exclusively in adults.In hereditary ichthyosis, usually at least one of the affected person's parents had the same dry, scaly skin as a child. It is inherited in an autosomal dominant fashion, meaning that each child of an affected parent has a 50-50 chance of having hereditary ichthyosis vulgaris.
Signs & Symptoms
The most common locations for ichthyosis vulgaris include:- Fronts (extensor surfaces) of the legs
- Backs (extensor surfaces) of the arms
- Scalp
- Back
- Forehead and cheeks, especially in younger children
The following areas tend to NOT be affected:
- Face
- Front of the neck
- Abdomen
- Folds in front of the elbows (flexural surfaces of the arms)
- Folds behind the knees (flexural surfaces of the legs)
- Poor nutrition
- Infections, such as leprosy or HIV/AIDS
- Glandular diseases, such as thyroid or parathyroid problems
- Sarcoidosis
- Cancer, such as lymphoma or multiple myeloma
- Use of certain medications, such as nicotinic acid, cimetidine, and clofazimine
Self-Care Guidelines
Hereditary ichthyosis vulgaris tends to improve after puberty, while acquired ichthyosis vulgaris requires treatment of the medical condition before it will improve.However, both conditions should improve by restoring moisture (hydration) to the skin. Creams and ointments are better moisturizers than lotions, and they work best when applied just after bathing, while the skin is still moist. The following over-the-counter products may be helpful:
- Preparations containing alpha-hydroxy acids such as glycolic acid or lactic acid
- Creams containing urea
- Over-the-counter cortisone cream (if the areas are itchy)
When to Seek Medical Care
If your child continues to have very dry, scaly skin despite twice daily application of an over-the-counter moisturizer, be sure to tell his/her pediatrician.Any adult who develops dry, scaly skin that is not improved by twice daily application of an over-the-counter moisturizer should see a physician for evaluation.
Treatments
To treat the dry, scaly skin of ichthyosis vulgaris, the physician may recommend a topical cream or lotion containing the following:- Prescription-strength alpha- or beta-hydroxy acids (glycolic acid, lactic acid, salicylic acid)
- Prescription-strength urea
- A retinoid medication such as tretinoin or tazarotene
- High concentrations of propylene glycol
- Isotretinoin, a very strong medication with many potential side effects, usually used in the treatment of severe, scarring acne
If acquired ichthyosis vulgaris is suspected, the doctor will likely attempt to determine the underlying medical condition or the medication that triggered its development. The prognosis for an adult with acquired ichthyosis vulgaris depends upon treating the underlying medical condition or discontinuing the triggering medication.
References
Bolognia, Jean L., ed. Dermatology, pp.775-781. New York: Mosby, 2003.
Freedberg, Irwin M., ed. Fitzpatrick's Dermatology in General Medicine. 6th ed, pp.486, 482, 2353. New York: McGraw-Hill, 2003.
Ichthyosis vulgaris in Adult