Angioma Serpiginosum

Updated: Jul 14, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Overview

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]

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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]

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Epidemiology

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.

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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.

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