eMedicine Specialties > Dermatology > Diseases of the Vessels

Angioma Serpiginosum

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Cris Jagar, MD, Staff Physician, Department of Psychiatry, Saint Vincent Catholic Medical Centers
Contributor Information and Disclosures

Updated: Jun 12, 2009

Introduction

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-Crocker3 gave this condition its name, angioma serpiginosum, in 1894. Frain-Bell4 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 lesions5,6 may be present.7

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.

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.

Clinical

History

Angioma serpiginosum, a rare vascular nevoid disorder due to ectatic dilation of capillaries in the papillary dermis, is found almost exclusively in females. In 2005, Sandhu and Gupta7 reported 2 rare cases—one with familial involvement and the other with an extensive distribution of lesions. Affected individuals tend to have grouped erythematous punctate lesions on the lower limbs or buttocks.

  • A port-wine stain may be the first evidence of this disorder, appearing during the first few months of life.
  • Years later, it may slowly enlarge, not by a uniform edge but rather by minute satellites ranging from copper-red to vividly red.
  • Satellites spread into circles and gradually coalesce, producing the irregular serpiginous pattern.
  • When the lesion resolves, it does so with a very superficial scar.
  • Lesions can be located anywhere on the body and have been reported in all areas except the palms and mucous membranes.7
  • Areas of predilection are the extremities, especially the lower extremities.
  • Patches are progressive and asymptomatic and rarely resolve.
  • Rarely, patches may be extensive in distribution.7
  • Numerous small, relatively well-demarcated, round-to-oval red lagoons may be visualized with dermoscopy, which can be beneficial in the diagnosis of angioma serpiginosum.8
  • Retinal involvement has been described.9
  • Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12.10 A 4-generation family with localized subepidermal telangiectasias following Blaschko lines (angioma serpiginosum) was described, with vascular streaks present at birth and that progressed slowly thereafter. Several family members had papillomatosis of the entire esophagus.

Physical

  • Angioma serpiginosum is composed of reddish-purple puncta that may be as large as 1 mm. They are usually found grouped on the lower extremities in a serpiginous pattern. Rarely, the sole may be involved.11  Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy.12
  • Angioma serpiginosum is variably compressible.
  • The lack of inflammation, hemorrhage, or hemosiderin pigmentation is characteristic.
  • Diascopic pressure applied to the lesion may produce only partial emptying, with some small tufts distended by purple venous blood remaining unchanged.
  • Dermoscopic examination shows multiple sharply demarcated red lagoons.13

Causes

  • Angioma serpiginosum may be familial, with autosomal dominant inheritance and variable penetrance.14

More on Angioma Serpiginosum

Overview: Angioma Serpiginosum
Differential Diagnoses & Workup: Angioma Serpiginosum
Treatment & Medication: Angioma Serpiginosum
Follow-up: Angioma Serpiginosum
References
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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. 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].

  9. 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].

  10. 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].

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

  12. 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].

  13. 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].

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

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

  16. 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].

  17. 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].

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

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

  20. Barker LP, Sachs PM. Angioma serpiginosum, a comparative study. Arch Dermatol. Dec 1965;92(6):613-20. [Medline].

  21. Chavaz P, Laugier P. [Angioma serpiginosum Hutchinson: ultrastructural study (author's transl)]. Ann Dermatol Venereol. 1981;108(5):429-36. [Medline].

  22. Kumakiri M, Katoh N, Miura Y. Angioma serpiginosum. J Cutan Pathol. Dec 1980;7(6):410-21. [Medline].

  23. Montgomery H, Bailey RJ. Angioma serpiginosum. Brit J Dermatol. 1935;47:456-63.

  24. Reymond JL, Stoebner P, Amblard P. [Acquired nevoid telangiectasia (author's transl)]. Dermatologica. 1979;159(6):489-94. [Medline].

  25. Stevenson JR, Lincoln CS Jr. Angioma serpiginosum. Arch Dermatol. Jan 1967;95(1):16-22. [Medline].

  26. Svendsen E, Olsen P. [Angioma serpiginosum. A diagnostic problem]. Tidsskr Nor Laegeforen. Jun 30 1985;105(19-21):1308-9. [Medline].

  27. Whyte MP. Angiokeratoma serpiginosum. Int J Dermatol. Dec 1978;17(10):793-8. [Medline].

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Further Reading

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Keywords

angioma serpiginosa, cutaneous vascular nevus, serpiginous nevus, vascular nevus

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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, UMDNJ-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, Saint Vincent Catholic Medical Centers
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-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.

CME Editor

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

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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