Elastoderma

Definition

Elastoderma is a rare condition that affects the skin. People affected by elastoderma generally have increased laxity of skin covering a specific area of the body.

Cause

• The exact underlying cause of elastoderma is currently unknown.
• It is thought to occur when there is an over-production of elastin in a specific area of skin.
• Elastin is a protein that is a vital component of skin and other connective tissues throughout the body.
• It serves many roles.
• For example, elastin provides strength and flexibility to connective tissues; give structural support to organs and tissues such as the heart, skin, and lungs; and helps the skin return to its original position when it is poked or pinched (recoil).
• It is unclear what causes this increase in elastin synthesis in people affected by elastoderma.

Inheritance

It generally occurs sporadically in people with no family history of the condition.

Signs and symptoms

• People affected by elastoderma generally have increased laxity of the skin that is localized to a specific area of the body.
• Skin is often described as lax when it hangs or sags in loose folds and is not stretchy.
• Affected areas of skin may appear wrinkled.
• Decreased recoil of the skin has also been reported.
• Although any part of the body can be affected, the skin of the neck and extremities (arms and legs, especially at the elbows and/or knees) are most commonly involved.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

100% of people have these symptoms

• Cutis laxa(Loose and inelastic skin)

80%-99% of people have these symptoms

• Papule
• Premature skin wrinkling
• Skin nodule

30%-79% of people have these symptoms

• Hyperesthesia

5%-29% of people have these symptoms

• Eczema
• Erysipelas

Diagnosis

• A diagnosis of elastoderma is generally made in people with characteristic signs and symptoms once other conditions that cause similar features have been ruled out.
• A skin biopsy may be recommended to confirm the diagnosis.

Treatment

• Due to the rarity of the condition, there is no standard therapy available for elastoderma.
• In some reported cases, the affected skin has been partially excised (removed).
• However, hyperlaxity of skin often returns following the surgery.

NIH genetic and rare disease info

• NIH info on Elastoderma

Elastoderma is a rare disease.

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