Elastosis Perforans Serpiginosa

What is Elastosis Perforans Serpiginosa?

Elastosis perforans serpiginosa is a rare skin disorder in which abnormal elastic tissue fiber passes from the inner layer of skin to the outer layer of skin. The inner layer of the skin is called the papillary dermis. The outer layer of the skin is called the epidermis.

When a person has elastosis perforans serpiginosa, the outer layer of the skin perceives abnormal elastic tissue to be a foreign object. When the elastic tissue is perceived as abnormal object, inflammation occurs.

Elastosis perforans serpiginosa most commonly appears during early adulthood (20-30 years) but may been seen in early childhood or late in life. It is more common in males than females.

Elastosis perforans serpiginosa is also called Lutz-Miescher’s syndrome.

Types of Elastosis Perforans Serpiginosa

There are three main forms of elastosis perforans serpiginosa.

1. Idiopathic elastosis perforans serpiginosa
2. Reactive elastosis perforans serpiginosa:
   • Often associated with Down syndrome, Ehler's Danlos syndrome and Marfan syndrome.
3. Drug-induced elastosis perforans serpiginosa:
   • Affects about 1 percent of people treated with D-penicillamine.

What causes Elastosis Perforans Serpiginosa?

Elastosis perforans serpiginosa is caused by several different things. It may be caused by a genetic abnormality or a side effect of medication.

What are the signs and symptoms of Elastosis Perforans Serpiginosa?

Common symptoms of elastosis perforans serpiginosa are:

• Cluster of small reddish bumps
  • The bumps are between 2 and 5 millimeters in diameter
  • The bumps usually has a circular or snake-like pattern
  • There may be a crusty or scaly plug in the center of the cluster.
  • The cluster usually appears on the back of the neck and on the arms. The cluster rarely appears on the face, legs and trunk.
• Itchy skin

Can Elastosis Perforans Serpiginosa be Treated?

Yes. The treatment options will depend on the cause and severity of the condition.