Angioma Serpiginosum Clinical Presentation
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
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 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. Late-onset ones may appear in a bandlike unilateral distribution on the chest. 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.
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. Reflectance confocal microcopy shows multiple dilated vacular spaces in the superficial dermis and a deeper vascular plexus parallel to the skin surface.
Retinal involvement has been described.
Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12. 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.
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. 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. Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy.
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. 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.
Hutchinson J. A peculiar form of serpiginous and infective naevoid disease. Arch Surg (London). 1889-1890. 1:275.
Hutchinson J. Infective angeioma, or naevus-lupus. Arch Surg (London). 1891-1892. 3:166-8.
Radcliffe-Crocker H. Diseases of the Skin. Philadelphia, Pa: Blakiston Press; 1893. 646.
Frain-BellW. Angioma serpiginosum. Br J Dermatol. 1957 Jul-Aug. 69(7-8):251-68. [Medline].
Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol. 2000 Feb. 42(2 Pt 2):384-5. [Medline].
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.
Sandhu K, Gupta S. Angioma serpiginosum: report of two unusual cases. J Eur Acad Dermatol Venereol. 2005 Jan. 19(1):127-8. [Medline].
Happle R. Capillary malformations: a classification using specific names for specific skin disorders. J Eur Acad Dermatol Venereol. 2015 Apr 10. [Medline].
Lombardi MP, Bulk S, Celli J, et al. Mutation update for the PORCN gene. Hum Mutat. 2011 Apr 5. [Medline].
Duman N, Ersoy-Evans S. Angioma serpiginosum: report of two cases suggesting type 1 mosaicism and proposal of adding it to the list of mosaic skin conditions. Int J Dermatol. 2015 Mar. 54 (3):e88-9. [Medline].
Savant SS, Das A, Kumar P, Hassan S. Late-onset Segmental Angioma Serpiginosum. Indian J Dermatol. 2016 Mar-Apr. 61 (2):226-7. [Medline].
Ilknur T, Fetil E, Akarsu S, Altiner DD, Ulukus C, Gunes AT. Angioma serpiginosum: dermoscopy for diagnosis, pulsed dye laser for treatment. J Dermatol. 2006 Apr. 33(4):252-5. [Medline].
Marks V, Tcheung WJ, Burton C 3rd, Selim A, Nelson K. Reflectance confocal microscopy features of angioma serpiginosum. Arch Dermatol. 2011 Jul. 147(7):878. [Medline].
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. 2004 Mar. 18(2):238-9. [Medline].
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. 2007 May. 15(5):543-7. [Medline].
Das D, Nayak CS, Tambe SA. Blaschko-linear angioma serpiginosum. Indian J Dermatol Venereol Leprol. 2016 May-Jun. 82 (3):335-7. [Medline].
Bayramgurler D, Filinte D, Kiran R. Angioma serpiginosum with sole involvement. Eur J Dermatol. 2008 Nov-Dec. 18(6):708-9. [Medline].
Mayer V, Maetzke J, Scharffetter-Kochanek K. [Punctate maculae on the back of the hands of a 44-year-old woman]. Hautarzt. 2009 May. 60(5):428-32. [Medline].
Kalisiak MS, Haber RM. Angioma serpiginosum with linear distribution: case report and review of the literature. J Cutan Med Surg. 2008 Jul-Aug. 12(4):180-3. [Medline].
Ghanadan A, Kamyab-Hesari K, Moslehi H, Abasi A. Dermoscopy of angioma serpiginosum: a case report. Int J Dermatol. 2014 Apr 16. [Medline].
Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum--familial incidence. Br J Dermatol. 1975 Dec. 93(6):701-6. [Medline].
Dammert K. Angiokeratosis naeviformis--a form of naevus telanggiectaticus lateralis (naevus flammeus). Dermatologica. 1965. 130:17-39. [Medline].
Mallo-Garcia S, Coto-Segura P, Galache-Osuna C, Santos-Juanes-Jimenez J. [Six cases of nevus oligemicus]. Actas Dermosifiliogr. 2008 May. 99(4):301-4. [Medline].
Vivehanantha S, Browne F, Bowen C, Brundler MA, Hughes J, Moss C, et al. A congenital smooth muscle hamartoma masquerading as a reticulate vascular naevus. Clin Exp Dermatol. 2013 Jun 13. [Medline].
Miura T, Yamamoto T. Pediatric linear scleroderma initially developed with angioma serpiginosum-like appearances. J Dermatol. 2015 May 11. [Medline].
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].
Madan V, August PJ, Ferguson JE. Pulsed-dye laser treatment of angioma serpiginosum. Clin Exp Dermatol. 2008 Dec 2. [Medline].
Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol. 1997 Apr. 136(4):631-2. [Medline].
Madan V, August PJ, Ferguson JE. Pulsed-dye laser treatment of angioma serpiginosum. Clin Exp Dermatol. 2009 Jul. 34(5):e186-8. [Medline].