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Elastosis Perforans Serpiginosum Clinical Presentation

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

History

In elastosis perforans serpiginosa (EPS), small papules erupt and are grouped in a confined area, eventually becoming serpiginous. The central core of each papule contains a compressed aggregate of fibrous material and cellular debris that, eventually, is disgorged to the surface, after which the papule subsides and disappears.

Elastosis perforans serpiginosa usually is asymptomatic but can be pruritic. Secondary lesions are vague scars or areas of roughness and hypopigmentation or hyperpigmentation that result after the primary lesions have subsided and disappeared.

The three general categories of elastosis perforans serpiginosa are idiopathic, reactive, and drug induced.

Idiopathic elastosis perforans serpiginosa

Familial groupings have implicated both dominant and recessive types of inheritance. Inciting events are not known.[1, 2] Probably, 65% (or more) of elastosis perforans serpiginosa cases are idiopathic.

Reactive elastosis perforans serpiginosa

This form of elastosis perforans serpiginosa is associated with other diseases (eg, Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, scleroderma, acrogeria, perforating granuloma annulare, pseudoxanthoma elasticum).

More than 1% of people with Down syndrome have elastosis perforans serpiginosa. One reported case of elastosis perforans serpiginosa was seen in Moyamoya disease, which is a progressive occlusive disease of the cerebral vessels. This patient also had Down syndrome, so it is unclear if the elastosis perforans serpiginosa association was with Moyamoya disease and could be a marker of elastosis perforans serpiginosa.[3]

Perforating diseases that may occur in conjunction with elastosis perforans serpiginosa include reactive perforating collagenosis, perforating folliculitis, pseudoxanthoma elasticum,[4] and Kyrle disease.[5] Sufficient similarities and overlapping features are seen to suspect them of being variants of elastosis perforans serpiginosa in these patients; however, elastosis perforans serpiginosa is not associated regularly with diabetes, while the other three diseases usually are associated with diabetes. Reactive types comprise 25-30% of cases of elastosis perforans serpiginosa.

Drug-induced elastosis perforans serpiginosa

D-penicillamine appears to be the only drug for which elastosis perforans serpiginosa is an adverse effect. This form of elastosis perforans serpiginosa occurs in approximately 1% of treated patients. Usually, long-term treatment for Wilson disease provokes the occasional case of elastosis perforans serpiginosa, but its use in the treatment of cystinuria and rheumatoid arthritis has had similar effects. The elastosis perforans serpiginosa eruption may appear 1 or more years after the start of treatment. This eruption often is symmetrical, widespread, and long lasting. It may appear at any time after treatment begins and usually resolves after withdrawal of the drug.

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Physical

Clinical features of elastosis perforans serpiginosa

Primary lesions are eruptive, dome-shaped, eventually umbilicated papules, measuring a few to several millimeters in diameter. Lesions are flesh colored to red. Usually, lesions are grouped in linear, arciform, circular, or serpiginous patterns, measuring several centimeters in diameter. New papules may appear in lines and figures that appear to migrate and enlarge peripherally.

Distribution of elastosis perforans serpiginosa

Any part of the skin may be affected. Most patients have only 1 area of skin involvement at a time, with the exception of Down syndrome patients, who typically have numerous active lesions. Frequently, these patients have symmetric extremity lesions. If multiple lesions are present, the figures may display a degree of anatomic symmetry.

Sites of elastosis perforans serpiginosa

The sites most commonly affected are the nape of the neck (70%), upper extremities (20%), face (11%), lower extremities (6%), and trunk (3%). One case of penile involvement was reported in 2003.[6] Another report from 2009 described a case induced by penicillamine therapy that occurred on the lip mucosa.[7, 8]

Elastosis perforans serpiginosa in an arciform pat Elastosis perforans serpiginosa in an arciform pattern on nape of neck.
Advancing serpiginous arrangement of elastosis per Advancing serpiginous arrangement of elastosis perforans serpiginosa papules with mild scarring in their wake.
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Causes

Elastic fibers are perceived by the skin to be abnormal and become the primary objects of an inflammatory attack. They may be abnormal as a result of genetic influence or penicillamine. Genetic abnormality does not explain the sporadic nature of elastosis perforans serpiginosa nor its spontaneous disappearance. Obviously, a secondary factor must be required to make elastic fibers provoke their own expulsion.

In reports and reviews of elastosis perforans serpiginosa, the swollen, broken, and deformed elastic fibers always are clumped in the papillary dermis, making transepidermal elimination convenient. This finding suggests that the unknown secondary factor that denatures cutaneous elastic fibers may have its source outside the body.

Heat, light, or a traumatic event can be at fault.

In penicillamine-induced cases,[9] proper cross-linkage formation has been observed to occur with the assistance of lysyl oxidase, which is a copper-dependent enzyme.[10]  Penicillamine is a copper chelator and may interfere with the function of lysyl oxidase and the proper construction of elastic fibers.

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Complications

Vascular or visceral rupture is possible with systemic elastosis perforans serpiginosa.

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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

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Acknowledgements

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

References
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  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. 1993 Dec. 32(12):879-81. [Medline].

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  5. 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.

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

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  8. 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. 2011 Apr 15. 17(4):3. [Medline].

  9. Liang J, Wang D, Xu J, Chen L. Two cases of D-penicillamine-induced elastosis perforans serpiginosa. Indian J Dermatol Venereol Leprol. 2016 Jul-Aug. 82 (4):452-4. [Medline].

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

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

  12. 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. 2000 Oct. 115(4):633-9. [Medline].

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

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

  16. Wang D, Liang J, Xu J, Chen L. Effective treatment of d-penicillamine induced elastosis perforans serpiginosa with ALA-PDT. Photodiagnosis Photodyn Ther. 2015 Mar. 12 (1):140-2. [Medline].

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

  18. 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. 2010 Jan-Feb. 14(1):38-42. [Medline].

  19. 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. 2010 Jan-Feb. 14(1):38-42. [Medline].

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