Are You Confident of the Diagnosis?
What to be alert for in the history
Angioma serpiginosum (AS) begins in first two decades of life. It is usually asymptomatic and occurs sporadically; some familial cases have been reported. It most commonly affects the extremities with extension possible over time. The incidence favors females over males.
Characteristic findings on physical examination
Deep red to purple punctate lesions in a cluster or sheet, which may form a serpiginous pattern, are seen on physical examination. They are nonpalpable with incomplete blanching. The extremities are most commonly affected. Distribution is initially unilateral; subsequent extension (both unilaterally and onto bilateral surfaces) is possible over time. The palms, soles, and mucous membranes are spared.
Expected results of diagnostic studies
Histopathology reveals dilated capillaries in the dermal papillae with no signs of inflammation.
Diagnosis confirmation
The differential diagnosis includes pigmented purpuric rashes. These are usually symptomatic, bilateral, and have evidence of inflammation on skin biopsy.
Who is at Risk for Developing Angioma serpiginosum?
This condition is rare, and often occurs sporadically, although some familial cases have been reported. Females are most commonly affected.
What is the Cause of Angioma serpiginosum?
Etiology
The etiology is unknown.
Pathophysiology
Dilated capillaries in the dermal papillae without inflammation
Systemic Implications and Complications
AS is asymptomatic. It can become a cosmetic concern.
Treatment Options
Treatment is usually unnecessary. It can be treated with pulsed dye laser.
Optimal Therapeutic Approach for Angioma serpiginosum
Treatment is usually unnecessary. Reassurance is key. If treatment is desired (eg, for cosmetic reasons), improvement can be achieved with pulsed dye laser.
Patient Management
Reassurance is key. Extension of lesions (both unilaterally and bilaterally) is possible. Treatment is usually unnecessary. Cases are rarely familial, and most often are sporadic.
Unusual Clinical Scenarios to Consider in Patient Management
Lesions may clinically appear similar to those seen in focal dermal hypoplasia of Goltz. A mutation of the PORCN gene is responsible for the Goltz syndrome but is not present in AS.
Late-onset AS has been reported.
What is the Evidence?
Baker, C, Kelly, R, Bolognia, JL, Jorizzo, JL, Rapini, RP. “Other Vascular Disorders. In Dermatology”. 2008. pp. 1615-1625. (An excellent, textbook review on AS and other vascular lesions; includes good clinical and pathologic photographs.)
Chen, J-H, Wang, K-H, Hu, C-H, Chiu, J-S. “Atypical angioma serpiginosum”. Yonsei Med J. vol. 49. 2008. pp. 509-13. (An unusual case of lower extremity AS in a 48-year-old woman whose lesions first started to appear in her early 40s.)
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