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Acral peeling skin syndrome

Acral peeling skin syndrome is a genetic skin disorder characterized by painless peeling of the top layer of skin

Prevalence

<1 / 1 000 000

< 331

US Estimated

< 514

Europe Estimated

Age of Onset

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ICD-10

D80.8

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

Acral peeling skin syndrome is a genetic skin disorder characterized by painless peeling of the top layer of skin. It is an autosomal recessive disorder characterized by lifelong peeling of the stratum corneum, and may be associated with pruritus, short stature, and anagen hair.
"Acral" refers to the fact that the peeling of the skin is most noticeable on the hands and feet of this state.

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5 Facts you should know

FACT

1

A genetic skin disorder characterized by painless peeling of the top layer of skin

FACT

2

The peeling is usually present from birth, but can appear later in childhood or early adulthood

FACT

3

Acral peeling skin syndrome can be caused by mutations in the TGM5 gene recessive pattern

FACT

4

Skin peeling is made worse by exposure to heat, humidity and other forms of moisture, and friction

FACT

5

There is no cure for acral peeling skin syndrome

Acral peeling skin syndrome is also known as...

Acral peeling skin syndrome

Acral PSS; Acral deciduous skin; Localized PSS

What’s your Rare IQ?

At what age is Acral peeling skin syndrome most often diagnosed?

Common signs & symptoms

Abnormal blistering of the skin

Blistering, generalized

Allergy

Erythema

Eczema

High hypermetropia

Severe farsightedness

Ichthyosis

Scaling skin

Flaking, peeling, scaly

Rare View

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Current treatments

There is no cure for acral peeling skin syndrome. Treatment is centered on preventing skin damage and addressing symptoms as they occur. Emollients are often used to reduce skin peeling. If blister develop, they may be lanced with a sterile needle. Light dressings should be applied to prevent infection. The condition may be made worse by hot temperatures, high humidity, and friction. Individuals with acral peeling skin syndrome should avoid these condition, when possible. Immersion in water should also be avoided.

Top Clinical Trials

At the time this analysis was conducted, there were no interventional clinical studies being conducted.

Top Treatments in Research

At the time this analysis was conducted, there were no treatments in development for this disease.

† Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 https://rarediseases.info.nih.gov