What is Elastosis Perforans Serpiginosa & How is it Treated?|Risk, Causes, Symptoms of Elastosis Perforans Serpiginosa

EPS or Elastosis perforans serpiginosa is a rarely occurring disorder of the skin where there is passage of abnormal fibre of elastic tissue starting from the inner skin layer (papillary dermis) to the outer skin layer (epidermis)^{(1, 2, 3)}. Elastosis perforans serpiginosa is characterized as small reddish bumps on the skin⁽¹⁾.

There are 3 known types of Elastosis Perforans Serpiginosa:

1. Idiopathic Elastosis Perforans Serpiginosa:

   This type of EPS or Elastosis perforans serpiginosa is of unknown origin and is thought to be hereditary.

2. Reactive Elastosis Perforans Serpiginosa:

   This type of EPS or Elastosis perforans serpiginosa is linked with other hereditary disorders like Marfan syndrome, Ehlers Danlos syndrome and Down syndrome.

3. Elastosis Perforans Serpiginosa Induced by Drugs:

   This type of Elastosis perforans serpiginosa involves about 1% of the population whose treatment has been done with D-penicillamine.

Elastosis perforans serpiginosa commonly develops in early adulthood; however, can also develop late in life or in early childhood. Males are more affected with Elastosis Perforans Serpiginosa than females⁽¹⁾.

The cause of Elastosis perforans serpiginosa is the perception of the outer skin layer of the abnormal elastic tissue as a foreign object and thus resulting in an inflammatory attack. The exact cause of development of the abnormal elastic tissue is not known⁽³⁾; however, outside elements, such as penicillamine; and genetic mutation, are thought to cause EPS or Elastosis perforans serpiginosa.^{(1, 4)}

Elastosis perforans serpiginosa is characterized as a bunch of reddish small bumps about 2 to 5 mm in width. These bumps of Elastosis perforans serpiginosa are commonly characterized in circular, linear or snake-like patterns. There may be a presence of central pit in each of the lesion, which can be filled by a scaly or crusty plug. Most of the times, Elastosis Perforans Serpiginosa has no symptoms; however, patient can experience itchiness.

Elastosis perforans serpiginosa more commonly develops on the posterior side of the neck, and lesser on one or both arms, face, legs and trunk.

The clinical appearance of elastosis perforans serpiginosa along with its characteristic histology as seen from skin biopsy displaying transepidermal elimination of elastic tissue is what helps with the diagnosis of this rare skin condition⁽⁶⁾.

As of now, elastosis perforans serpiginosa has no cure. In most of the cases, this condition can spontaneously resolve on its own after some years⁽⁵⁾.

References: