Elastosis Perforans Serpiginosum

Updated: Jul 15, 2016
  • Author: Elizabeth A Liotta, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Elastosis perforans serpiginosa (EPS) is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis through the epidermis (transepithelial elimination). Elastosis perforans serpiginosa may be distinguished morphologically and histologically from other cutaneous diseases.

The first recognizable description of elastosis perforans serpiginosa was provided by Fischer in 1927 but was offered as an example of Kyrle disease. Jones and Smith also described elastosis perforans serpiginosa in 1947 but mistook it for porokeratosis of Mibelli. In 1953, Lutz recognized the features of elastosis perforans serpiginosa as those of an unknown disease and termed the condition keratosis follicularis serpiginosa. Miescher believed the condition was unique and termed it elastoma intrapapillare perforans verruciform.

Elastosis perforans serpiginosa is a disease of connective tissue, possibly of autoantigenic etiology, that occurs in the following three forms:

  • Idiopathic elastosis perforans serpiginosa: The cause of idiopathic elastosis perforans serpiginosa is unknown; genetic predisposition is possible.
  • Reactive elastosis perforans serpiginosa: This form is associated regularly with systemic, inherited, fibrous tissue abnormalities, such as Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, scleroderma, and pseudoxanthoma elasticum.
  • Drug-induced elastosis perforans serpiginosa: This form is caused by the pharmacologic effect of D-penicillamine and occurs in approximately 1% of patients treated with the drug.
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Pathophysiology

Elastosis perforans serpiginosa (EPS) may be a form of granulomatous inflammation that displays an unusual method for removing elastic tissue from the area of involvement.

Skin responds to threats to its integrity by foreign materials, such as bacteria, parasites, fungi, and misplaced native tissue elements (eg, hair), silica, beryllium, suture materials, through granulomatous inflammation.

The first stage of the granulomatous inflammation is the destruction of normal connective tissue. Then, an attack on the foreign material in the region ensues with waves of phagocytic histiocytes, often clumped into giant cells, moving in to engulf the foreign substances and debris. These phagocytic histiocytes carry away the foreign substances and debris, usually via vascular channels, but in the perforating diseases, via direct extrusion. The repair process follows close behind, with neovascularization and fibrosis. The duration of such a project is highly variable, often taking years to complete.

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Epidemiology

Frequency

Elastosis perforans serpiginosa is a rare condition. No geographic preferences are apparent for elastosis perforans serpiginosa.

Race

No racial differences have been reported for elastosis perforans serpiginosa.

Sex

Male-to-female ratio for elastosis perforans serpiginosa is approximately 4:1.

Age

Elastosis perforans serpiginosa usually appears during the second decade of life, but it may be seen in early childhood or late in life.

Duration

Elastosis perforans serpiginosa lasts a few years; however, in elastosis perforans serpiginosa cases associated with Down syndrome, elastosis perforans serpiginosa is a more prolonged and generalized eruption.

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Prognosis

In most cases, cutaneous elastosis perforans serpiginosa is of cosmetic consequence only. A rare systemic version is fatal, in which abnormal elastic tissue is found in the walls of ruptured blood vessels and viscera.

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