Elastosis Perforans Serpiginosum 

  • Author: Elizabeth A Liotta, MD; Chief Editor: William D James, MD   more...
 
Updated: Jan 17, 2012
 

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 3 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

United States

Elastosis perforans serpiginosa is a rare condition.

International

No geographic preferences are apparent for elastosis perforans serpiginosa.

Mortality/Morbidity

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.

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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Contributor Information and Disclosures
Author

Elizabeth A Liotta, MD  Chief Dermatologist and Sole Proprietor, Integrated Skin Care Centers

Elizabeth A Liotta, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Willard Steck, MD, FACP, to the development and writing of this article.

References

  1. Langeveld-Wildschut EG, Toonstra J, van Vloten WA, Beemer FA. Familial elastosis perforans serpiginosa. Arch Dermatol. Feb 1993;129(2):205-7. [Medline].

  2. Rios-Buceta L, Amigo-Echenagusia A, Sols-Candelas M, Fraga-Fernandez J, Fernandez-Herrera J. Elastosis perforans serpiginosa with simultaneous onset in two sisters. Int J Dermatol. Dec 1993;32(12):879-81. [Medline].

  3. Espinosa PS, Baumann RJ, Vaishnav AG. Elastosis perforans serpiginosa, Down syndrome, and moyamoya disease. Pediatr Neurol. Apr 2008;38(4):287-8. [Medline].

  4. Abe R, Murase S, Nomura Y, Natuga K, Tateishi Y, Tomita Y, et al. Aquired perforating dermatosis appearing as elastosis perforans serpiginosa and perforating folliculitis. Clin Exp Dermatol. Aug/2008;33:653-4.

  5. Roest MA, Ratnavel R. Elastosis perforans serpiginosa of the penis. BJU Int. Mar 2003;91(4):427. [Medline].

  6. Lewis BK, Chern PL, Stone MS. Penicillamine-induced elastosis of the mucosal lip. J Am Acad Dermatol. Apr 2009;60(4):700-3. [Medline].

  7. Atzori L, Pinna AL, Pau M, Aste N. D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature. Dermatol Online J. Apr 15 2011;17(4):3. [Medline].

  8. Hashimoto K, McEvoy B, Belcher R. Ultrastructure of penicillamine-induced skin lesions. J Am Acad Dermatol. Mar 1981;4(3):300-15. [Medline].

  9. Fujimoto N, Akagi A, Tajima S, et al. Expression of the 67-kDa elastin receptor in perforating skin disorders. Br J Dermatol. Jan 2002;146(1):74-9. [Medline].

  10. Fujimoto N, Tajima S, Ishibashi A. Elastin peptides induce migration and terminal differentiation of cultured keratinocytes via 67 kDa elastin receptor in vitro: 67 kDa elastin receptor is expressed in the keratinocytes eliminating elastic materials in elastosis perforans serpiginosa. J Invest Dermatol. Oct 2000;115(4):633-9. [Medline].

  11. Ratnavel RC, Norris PG. Penicillamine-induced elastosis perforans serpiginosa treated successfully with isotretinoin. Dermatology. 1994;189(1):81-3. [Medline].

  12. Outland JD, Brown TS, Callen JP. Tazarotene is an effective therapy for elastosis perforans serpiginosa. Arch Dermatol. Feb 2002;138(2):169-71. [Medline].

  13. Kelly SC, Purcell SM. Imiquimod therapy for elastosis perforans serpiginosa. Arch Dermatol. Jul 2006;142(7):829-30. [Medline].

  14. Tuyp EJ, McLeod WA. Elastosis perforans serpiginosa: treatment with liquid nitrogen. Int J Dermatol. Nov 1990;29(9):655-6. [Medline].

  15. Humphrey S, Hemmati I, Randhawa R, Crawford RI, Hong CH. Elastosis perforans serpignosa: treatment with liquid nitrogen cryotherapy and review of the literature. J Cutan Med Surg. Jan-Feb 2010;14(1):38-42. [Medline].

  16. Saxena M, Tope WD. Response of elastosis perforans serpiginosa to pulsed CO2, Er:YAG, and dye lasers. Dermatol Surg. Jun 2003;29(6):677-8. [Medline].

  17. Kaufman AJ. Treatment of elastosis perforans serpiginosa with the flashlamp pulsed dye laser. Dermatol Surg. Nov 2000;26(11):1060-2. [Medline].

  18. [Guideline] Advisory Committee on Epidemiology. Guidelines for control of meningococcal disease. The Advisory Committee on Epidemiology. Can Dis Wkly Rep. Nov 9 1991;17(45):245-50. [Medline].

  19. Allen P. On first looking into Pernkopf's Atlas: some further comments. Arch Dermatol. Feb 2002;138(2):266-7. [Medline].

  20. Iozumi K, Nakagawa H, Tamaki K. Penicillamine-induced degenerative dermatoses: report of a case and brief review of such dermatoses. J Dermatol. Jul 1997;24(7):458-65. [Medline].

  21. London ID, Givhan EG, Garrick J, Mehregan AH. Elastosis perforans serpiginosa with systemic involvement. South Med J. Feb 1974;67(2):225-7. [Medline].

  22. Mehregan AH. Elastosis perforans serpiginosa: a review of the literature and report of 11 cases. Arch Dermatol. Apr 1968;97(4):381-93. [Medline].

  23. Morgan MB, Truitt CA, Taira J, Somach S, Pitha JV, Everett MA. Fibronectin and the extracellular matrix in the perforating disorders of the skin. Am J Dermatopathol. Apr 1998;20(2):147-54. [Medline].

  24. Patterson JW. The perforating disorders. J Am Acad Dermatol. Apr 1984;10(4):561-81. [Medline].

  25. Rubio FA, Robayna G, Borbujo J, De Lucas R, Casado M. An unusual presentation of elastosis perforans serpiginosa. Pediatr Dermatol. Sep-Oct 1996;13(5):435-6. [Medline].

  26. Siragusa M, Romano C, Cavallari V, Schepis C. Localized elastosis perforans serpiginosa in a boy with Down syndrome. Pediatr Dermatol. May-Jun 1997;14(3):244-6. [Medline].

 
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Elastosis perforans serpiginosa in an arciform pattern on nape of neck.
Advancing serpiginous arrangement of elastosis perforans serpiginosa papules with mild scarring in their wake.
Histologic section of elastosis perforans serpiginosa stained with hematoxylin and eosin. Connective tissue fibers and cellular debris are extruded through the epidermis via a spiraling path.
Cross-section of a nidus of fibers and debris of elastosis perforans serpiginosa in transit through the epidermis, stained with hematoxylin and eosin. Elastic fibers are red.
Connective tissue and debris of elastosis perforans serpiginosa emerging through the epidermis toward the surface, and elastic fibers in the nearby papillary dermis. The stain is a variation on acid orcein-Giemsa. Elastic fibers are black.
 
 
 
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