Elastosis perforans serpiginosa

What is elastosis perforans serpiginosa?

Elastosis perforans serpiginosa (EPS) is a rare skin disorder in which abnormal elastic tissue fibre passes from the papillary dermis (inner layer of skin) to the epidermis (outer layer of skin). This is described as transepithelial elimination and results in a group of small reddish bumps.

Three forms of EPS have been identified:

• Idiopathic EPS: unknown origin, possibly genetic
• Reactive EPS: associated with other inherited disorders such as Down syndrome, Ehlers Danlos syndrome and Marfan syndrome
• Drug-induced EPS: affects 1% of people treated with D-penicillamine

Who gets EPS?

EPS most commonly appears during early adulthood (20-30 years) but may been seen in early childhood or late in life. It appears to be more common in males than females (ratio 4:1).

What causes EPS?

EPS occurs because the epidermis (the outer layer of the skin) perceives abnormal elastic tissue to be a foreign object, thus responding through an inflammatory attack. The cause of abnormal elastic tissue is unknown but may be from a genetic mutation or external factors such as penicillamine.

What are the signs and symptoms?

EPS presents as a cluster of small reddish bumps 2-5 mm in diameter, often grouped in linear, circular or serpiginous (snake-like) patterns. Each lesion may have a central pit, which is sometimes filled by a crusty or scaly plug. Usually there are no symptoms but sometimes EPS is itchy.

EPS usually arises on the back of the neck (70%) and less frequently on one or both arms (20%), face (11%), legs (6%) and trunk (3%).

Elastosis perforans serpiginosa

How is the diagnosis made?

The diagnosis of elastosis perforans serpiginosa is made by its clinical appearance and by the characteristic histology changes seen on skin biopsy, showing transepidermal elimination of elastic tissue.

What treatment is available?

There is no cure for EPS. Usually it spontaneously resolves without complications after a few years.