Elastosis Perforans Serpiginosum Treatment & Management

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

Medical Care

Little medical treatment is required for elastosis perforans serpiginosa (EPS), except to monitor for internal vascular complications.

  • Although many medications have been tried, no uniformly effective medications have been reported.
  • A few reports suggest benefit from isotretinoin, but little confirmation is available.[11]
  • If pruritus is present, symptomatic therapy with camphor-containing and/or menthol-containing products is beneficial.
  • A 2002 report described effective treatment with topical tazarotene 0.1% gel taken daily at bedtime after 2 months of treatment in 2 patients. The lesions were noted to flare upon discontinuation of the therapy.[12]
  • A 2006 case study described imiquimod as an effective treatment after 10 weeks of therapy.[13]
  • Cryotherapy has achieved some mild improvement in some patients.[14]
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Surgical Care

  • Destructive methods appear to do more harm than good, often resulting in scarring worse than that expected from spontaneous healing.
  • Freezing with liquid nitrogen may have helped several patients.[15]
  • Treatment with some laser techniques may have been modestly helpful in one patient with idiopathic elastosis perforans serpiginosa.[16]
  • Treatment with flashlight pulsed dye laser has appeared to be beneficial in one reported case of elastosis perforans serpiginosa in a patient with Down syndrome.[17]
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Activity

Avoid skin irritants.

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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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