Angioma Serpiginosum 

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD   more...
 
Updated: May 2, 2011
 

Background

Jonathan Hutchinson described a teenage girl with a "very peculiar condition of serpiginous or infective nevus" in 1890.[1] He noted that although nevi may increase in size and number early in life, it is unusual for them to continue to spread, as in his patient.[2] Radcliffe-Crocker[3] gave this condition its name, angioma serpiginosum, in 1894. Frain-Bell[4] presented 11 patients with this disorder in 1957, clearly distinguishing it from chronic pigmentary purpuras and other disorders. Rarely, familial involvement or an extensive distribution of lesions[5, 6] may be present.[7]

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Pathophysiology

Angioma serpiginosum is an uncommon cutaneous vascular nevus of superficial capillaries characterized by minute puncta in clusters or in a linear array (a serpiginous pattern). These puncta result from a congenital hyperplasia or ectasia of preexisting superficial dermal capillaries, which may ultimately disappear (probably as a result of thrombosis). Electron microscopic findings have supported the view that these lesions are due to a vascular anomaly rather than a simple telangiectasia.

PORCN gene mutations or deletions have been reported in angioma serpiginosum.[8]

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Epidemiology

Frequency

United States

Angioma serpiginosum is innocuous and thus is rarely reported, although it probably is not as unusual as current literature would suggest.

Mortality/Morbidity

No deaths have been reported from angioma serpiginosum. The only sources of possible morbidity arise from minor bleeding during treatment of the lesions.

Race

No increased frequency is observed in any particular racial group. Rarely, as reported by Sandhu and Gupta in 2005,[7] it may be familial.

Sex

Approximately 90% of affected patients are women.

Age

Eighty percent of patients are affected before age 20 years, and the condition has been described at birth in some cases.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Cris Jagar, MD  Staff Physician, Department of Psychiatry, Trinitas Regional Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Mark W Cobb, MD  Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

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

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

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

References

  1. Hutchinson J. A peculiar form of serpiginous and infective naevoid disease. Arch Surg (London). 1889-1890;1:275.

  2. Hutchinson J. Infective angeioma, or naevus-lupus. Arch Surg (London). 1891-1892;3:166-8.

  3. Radcliffe-Crocker H. Diseases of the Skin. Philadelphia, Pa: Blakiston Press; 1893:646.

  4. Frain-BellW. Angioma serpiginosum. Br J Dermatol. Jul-Aug 1957;69(7-8):251-68. [Medline].

  5. Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):384-5. [Medline].

  6. Wise F, Pollitzer S. Angioma serpiginosum (infective angioma of Hutchinson), with a report of a very extensive case. J Cutan Dis. 1913;31:725-39, 916-41.

  7. Sandhu K, Gupta S. Angioma serpiginosum: report of two unusual cases. J Eur Acad Dermatol Venereol. Jan 2005;19(1):127-8. [Medline].

  8. Lombardi MP, Bulk S, Celli J, et al. Mutation update for the PORCN gene. Hum Mutat. Apr 5 2011;[Medline].

  9. Ilknur T, Fetil E, Akarsu S, Altiner DD, Ulukus C, Gunes AT. Angioma serpiginosum: dermoscopy for diagnosis, pulsed dye laser for treatment. J Dermatol. Apr 2006;33(4):252-5. [Medline].

  10. Erbagci Z, Erbagci I, Erkilic S, Bekir N. Angioma serpiginosum with retinal involvement in a male: a possible aetiological role of continuous cold exposure. J Eur Acad Dermatol Venereol. Mar 2004;18(2):238-9. [Medline].

  11. Blinkenberg EO, Brendehaug A, Sandvik AK, Vatne O, Hennekam RC, Houge G. Angioma serpiginosum with oesophageal papillomatosis is an X-linked dominant condition that maps to Xp11.3-Xq12. Eur J Hum Genet. May 2007;15(5):543-7. [Medline].

  12. Bayramgurler D, Filinte D, Kiran R. Angioma serpiginosum with sole involvement. Eur J Dermatol. Nov-Dec 2008;18(6):708-9. [Medline].

  13. Mayer V, Maetzke J, Scharffetter-Kochanek K. [Punctate maculae on the back of the hands of a 44-year-old woman]. Hautarzt. May 2009;60(5):428-32. [Medline].

  14. Kalisiak MS, Haber RM. Angioma serpiginosum with linear distribution: case report and review of the literature. J Cutan Med Surg. Jul-Aug 2008;12(4):180-3. [Medline].

  15. Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum--familial incidence. Br J Dermatol. Dec 1975;93(6):701-6. [Medline].

  16. Dammert K. Angiokeratosis naeviformis--a form of naevus telanggiectaticus lateralis (naevus flammeus). Dermatologica. 1965;130:17-39. [Medline].

  17. Mallo-Garcia S, Coto-Segura P, Galache-Osuna C, Santos-Juanes-Jimenez J. [Six cases of nevus oligemicus]. Actas Dermosifiliogr. May 2008;99(4):301-4. [Medline].

  18. Polla LL, Tan OT, Garden JM, Parrish JA. Tunable pulsed dye laser for the treatment of benign cutaneous vascular ectasia. Dermatologica. 1987;174(1):11-7. [Medline].

  19. Madan V, August PJ, Ferguson JE. Pulsed-dye laser treatment of angioma serpiginosum. Clin Exp Dermatol. Dec 2 2008;[Medline].

  20. Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol. Apr 1997;136(4):631-2. [Medline].

  21. Madan V, August PJ, Ferguson JE. Pulsed-dye laser treatment of angioma serpiginosum. Clin Exp Dermatol. Jul 2009;34(5):e186-8. [Medline].

 
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