Angioma Serpiginosum 

Updated: May 29, 2020
Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD 

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]

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]

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.

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.

 

Presentation

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.

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.[12] 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.[13, 14, 15] Reflectance confocal microcopy shows multiple dilated vacular spaces in the superficial dermis and a deeper vascular plexus parallel to the skin surface.[16]

Retinal involvement has been described.[17]

Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12.[18] 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.[19]

Physical Examination

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.[20] Angioma serpiginosum is composed of reddish-purple puncta that may be as large as 1 mm. See the image below.

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

They are usually found grouped on the lower extremities in a serpiginous pattern. Rarely, the sole may be involved.[21] Punctate erythematous maculae on the backs of the hands, arms, and shoulders may appear following a pregnancy.[22] It may be evident in a patchy and blaschkoid distribution.[23]

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.[24, 25] 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.[26]

 

DDx

Diagnostic Considerations

Also consider the following:

  • Schamberg disease

  • Majocchi disease

  • Pigmented purpuric lichenoid eruption of Gougerot and Blum

  • Goltz syndrome post-zygotic mosaicism[27]

  • Nevus flammeus[28] (see Capillary Malformation)

  • Unilateral nevoid telangiectasia[15]

Nevus oligemicus is an uncommon hamartoma characterized by selective vasoconstriction of the deep dermal vascular plexus with respect to the superficial one. It is evident as fixed, acquired, asymptomatic, livid, erythematous macules that are cold to touch compared with surrounding skin.[29]

A linear reticulate, reddish-purple plaque on the buttock extending to the popliteal fossa showed a striking resemblance to a vascular lesion and was labelled a reticulate vascular nevus; it was observed in association with a congenital smooth muscle hamartoma.[30]

Pediatric linear scleroderma may have a clinical resemblance to angioma serpiginosum.[31]

Differential Diagnoses

 

Workup

Imaging Studies

Imaging studies are not usually necessary to evaluate this skin lesion and are indicated only if atypical findings are present and the diagnosis is unclear.

Procedures

A biopsy helps confirm the diagnosis.

Histologic Findings

The overlying epidermis is normal. The dermal papilla and subpapillary regions of the dermis show dilated capillaries with a thickening of the capillary walls. No inflammatory changes, hemorrhage, or hemosiderin depositions are present. Ultrastructural analysis of several lesions has shown that some thickening of vessel walls may occur from a heavy precipitate of basement membrane–like, fine fibrillar material mixed with thin collagen fibers. Some of these dilated capillaries show slitlike protrusions of lamina into the endothelial lining.

 

Treatment

Medical Care

This vascular lesion does not require medical care.

Surgical Care

Electrolysis or laser surgery of an individual lesion may be beneficial.[32] Good cosmetic results can be achieved with a tunable pulse dye laser by selective photothermolysis of the vascular ectasias.[22, 33, 34] With the tunable pulse dye laser, good-to-excellent results may be achieved in four or fewer visits.[35]

Related articles include Laser Treatment of Acquired and Congenital Vascular Lesions and Laser Treatment of Benign Pigmented Lesions.

Consultations

Consult a dermatologist for evaluation of the lesions and for possible treatment options. Consult a dermatopathologist for evaluation of the biopsy specimen.

 

Medication

Medication Summary

These skin lesions do not require pharmacologic therapy.