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Angioma Serpiginosum Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
Updated: Jun 22, 2016


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.

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] Late-onset ones may appear in a bandlike unilateral distribution on the chest.[11] 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.[12] Reflectance confocal microcopy shows multiple dilated vacular spaces in the superficial dermis and a deeper vascular plexus parallel to the skin surface.[13]

Retinal involvement has been described.[14]

Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12.[15] 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. Isolated cases of Blaschko-linear angioma serpiginosum have also been described.[16]



Angioma serpiginosum usually begins before puberty, tending to affect the lower limbs and buttocks. However, onset later in life and on other sites such as breast have been described.[17] 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.[18] Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy.[19]

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.[20] These multiple small, relatively well-demarcated, round-to-oval red lagoons may be associated with comma, hairpinlike vessels and patchy pigmentation dispersed through the background.[21]



Angioma serpiginosum may be familial, with autosomal dominant inheritance and variable penetrance.[22]

Contributor Information and Disclosures

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

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

Disclosure: Nothing to disclose.


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

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate 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, Texas Dermatological Society

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, American Society of Dermatopathology

Disclosure: Nothing to disclose.

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