AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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-Crocker³ gave this condition its name, angioma serpiginosum, in 1894. Frain-Bell⁴ 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

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.

Frequency

United States

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

Mortality/Morbidity

No deaths have been reported from angioma serpiginosum. The only sources of possible morbidity arise from minor bleeding during treatment of the lesions.

Race

No increased frequency is observed in any particular racial group. Rarely, as reported by Sandhu and Gupta in 2005⁷, it may be familial.

Sex

Approximately 90% of affected patients are women.

Age

Eighty percent of patients are affected before age 20 years, and the condition has been described at birth in some cases.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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 Gupta⁷ 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
• 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.⁸
• Retinal involvement has been described.⁹
• Angioma serpiginosum with esophageal papillomatosis has been described as an X-linked dominant condition that maps to Xp11.3-Xq12.¹⁰ 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.

Physical

• 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.
• It 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.

Causes

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

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Schamberg disease
Majocchi disease
Pigmented purpuric lichenoid eruption of Gougerot and Blum
Nevus flammeus¹¹ (see Capillary Malformation)

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.¹²

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

This vascular lesion does not require medical care.

Surgical Care

Electrolysis or laser surgery of an individual lesion may be beneficial.¹³ Good cosmetic results can be achieved with a tunable pulse dye laser by selective photothermolysis of the vascular ectasias.

Related eMedicine articles include Laser Treatment of Acquired and Congenital Vascular Lesions and Laser Treatment of Benign Pigmented Lesions, Additionally, the Medscape Aesthetic Medicine Resource Center may be of interest.

Consultations

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

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

These skin lesions do not require pharmacologic therapy.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Prognosis

• 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.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Misdiagnosis of angioma serpiginosum can delay effective treatment.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

1. Hutchinson J. A peculiar form of serpiginous and infective naevoid disease. Arch Surg (London). 1889-1890;1:275.
2. Hutchinson J. Infective angeioma, or naevus-lupus. Arch Surg (London). 1891-1892;3:166-8.
3. Radcliffe-Crocker H. Diseases of the Skin. Philadelphia, Pa: Blakiston Press; 1893:646.
4. Frain-Bell W. Angioma serpiginosum. Br J Dermatol. Jul-Aug 1957;69(7-8):251-68. [Medline].
5. Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):384-5. [Medline].
6. 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.
7. Sandhu K, Gupta S. Angioma serpiginosum: report of two unusual cases. J Eur Acad Dermatol Venereol. Jan 2005;19(1):127-8. [Medline].
8. Ilknur T, Fetil E, Akarsu S, Altiner DD, Ulukus C, Gunes AT. Angioma serpiginosum: dermoscopy for diagnosis, pulsed dye laser for treatment. J Dermatol. Apr 2006;33(4):252-5. [Medline].
9. 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. Mar 2004;18(2):238-9. [Medline].
10. 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. May 2007;15(5):543-7. [Medline].
11. Dammert K. Angiokeratosis naeviformis--a form of naevus telanggiectaticus lateralis (naevus flammeus). Dermatologica. 1965;130:17-39. [Medline].
12. Mallo-García S, Coto-Segura P, Galache-Osuna C, Santos-Juanes-Jiménez J. [Six cases of nevus oligemicus.]. Actas Dermosifiliogr. May 2008;99(4):301-4. [Medline].
13. 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].
14. Barker LP, Sachs PM. Angioma serpiginosum, a comparative study. Arch Dermatol. Dec 1965;92(6):613-20. [Medline].
15. Chavaz P, Laugier P. [Angioma serpiginosum Hutchinson: ultrastructural study (author's transl)]. Ann Dermatol Venereol. 1981;108(5):429-36. [Medline].
16. Kumakiri M, Katoh N, Miura Y. Angioma serpiginosum. J Cutan Pathol. Dec 1980;7(6):410-21. [Medline].
17. Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol. Apr 1997;136(4):631-2. [Medline].
18. Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum--familial incidence. Br J Dermatol. Dec 1975;93(6):701-6. [Medline].
19. Montgomery H, Bailey RJ. Angioma serpiginosum. Brit J Dermatol. 1935;47:456-63.
20. Reymond JL, Stoebner P, Amblard P. [Acquired nevoid telangiectasia (author's transl)]. Dermatologica. 1979;159(6):489-94. [Medline].
21. Stevenson JR, Lincoln CS Jr. Angioma serpiginosum. Arch Dermatol. Jan 1967;95(1):16-22. [Medline].
22. Svendsen E, Olsen P. [Angioma serpiginosum. A diagnostic problem]. Tidsskr Nor Laegeforen. Jun 30 1985;105(19-21):1308-9. [Medline].
23. Whyte MP. Angiokeratoma serpiginosum. Int J Dermatol. Dec 1978;17(10):793-8. [Medline].

Article Last Updated: May 1, 2008