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] Hereditary angioma serpiginosusm is best classified as a type of capillary malformation that does not represent a nevus, a category that includes X-linked angiokeratoma corporis diffusum (Fabry disease), autosomal dominant angiokeratoma corporis diffusum, hereditary hemorrhagic telangiectasia, and the salmon patch.^[8]

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.^[9]

Angioma serpiginosum may represent type 1 mosaicism.^[10]

Etiology

Angioma serpiginosum may be familial, with autosomal dominant inheritance and variable penetrance.^[11]

Frequency

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

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. The condition has been described at birth in some cases.

Prognosis

No deaths have been reported from angioma serpiginosum. The only sources of possible morbidity arise from minor bleeding during treatment of the lesions. This condition is slowly progressive despite long periods of relative stability. Spontaneous resolution is unusual and occurs late in the course, if at all. Good cosmetic results can be achieved with treatment. These lesions have no association with systemic disease.

Clinical Presentation

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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. [QxMD MEDLINE Link].
Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol. 2000 Feb. 42(2 Pt 2):384-5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Happle R. Capillary malformations: a classification using specific names for specific skin disorders. J Eur Acad Dermatol Venereol. 2015 Apr 10. [QxMD MEDLINE Link].
Lombardi MP, Bulk S, Celli J, et al. Mutation update for the PORCN gene. Hum Mutat. 2011 Apr 5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum--familial incidence. Br J Dermatol. 1975 Dec. 93(6):701-6. [QxMD MEDLINE Link].
Savant SS, Das A, Kumar P, Hassan S. Late-onset Segmental Angioma Serpiginosum. Indian J Dermatol. 2016 Mar-Apr. 61 (2):226-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Sinha P, Singh PY, Sood A, Bharadwaj R. Blaschkoid Angioma Serpiginosum: A Dermoscopic Diagnosis. Indian Dermatol Online J. 2018 Mar-Apr. 9 (2):127-129. [QxMD MEDLINE Link].
Villela-Segura U. Dermoscopy as an Important Tool for Differentiating Unilateral Nevoid Telangiectasia and Angioma Serpiginosum. Dermatol Pract Concept. 2019 Oct. 9 (4):306-307. [QxMD MEDLINE Link].
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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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Das D, Nayak CS, Tambe SA. Blaschko-linear angioma serpiginosum. Indian J Dermatol Venereol Leprol. 2016 May-Jun. 82 (3):335-7. [QxMD MEDLINE Link].
Mukherjee S, Salphale P, Singh V. Late onset angioma serpiginosum of the breast with co-existing cherry angioma. Indian Dermatol Online J. 2014 Jul. 5(3):316-9. [QxMD MEDLINE Link]. [Full Text].
Bayramgurler D, Filinte D, Kiran R. Angioma serpiginosum with sole involvement. Eur J Dermatol. 2008 Nov-Dec. 18(6):708-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Sancheti K, Das A, Podder I, Gharami RC. Angioma Serpiginosum in a Patchy and Blaschkoid Distribution: A Rare Condition with an Unconventional Presentation. Indian J Dermatol. 2016 Sep-Oct. 61 (5):570-2. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Ankad BS, Arora P, Sardana K, Bhardwaj M. Differentiation of acquired port wine stain and angioma serpiginosum: a dermoscopic perspective. Int J Dermatol. 2019 Mar. 58 (3):e62-e64. [QxMD MEDLINE Link].
Dhanta A, Taneja G, Hazarika N, Joshi P. Clinicopathological and Dermoscopic Features in Blashkoid Angioma Serpiginosum. Dermatol Pract Concept. 2021 Jan. 11 (1):e2021107. [QxMD MEDLINE Link].
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Media Gallery

Angioma serpiginosum. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/vascular/ang-serpig2.jpg).

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Angioma Serpiginosum

Background

Pathophysiology

Etiology

Epidemiology

Frequency

Race

Sex

Age

Prognosis

References

Tables

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