Angioma Serpiginosum Clinical Presentation

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

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 Gupta[7] 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. Note the following:

  • 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.[9]
  • Retinal involvement has been described.[10]
  • Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12.[11] 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.
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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.[12] Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy.[13]

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.[14]

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Causes

Angioma serpiginosum may be familial, with autosomal dominant inheritance and variable penetrance.[15]

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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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