What are the other Names for this Condition? (Also known as/Synonyms)

• Autosomal Dominant Angioma Serpiginosum
• Sporadic Angioma Serpiginosum
• X-Linked Angioma Serpiginosum

What is Angioma Serpiginosum? (Definition/Background Information)

• Angioma Serpiginosum (AS) is a skin condition that manifests as a collection of small blood vessels underneath the skin in a wavy or ring-like pattern, typically on the buttocks and legs
• The condition is generally noted in children with a vast majority involving girls. The cause of formation of Angioma Serpiginosum is generally unknown; although some cases are believed to be inherited, a majority occur sporadically
• The lesions characteristic of Angioma Serpiginosum are typically asymptomatic and benign that usually require no treatment. The outcomes are usually good, although persistence of the lesions may be noted

Who gets Angioma Serpiginosum? (Age and Sex Distribution)

• Angioma Serpiginosum is a rare skin disorder. The presentation of symptoms may occur between early childhood and teenage, usually before the individual reaches 20 years of age. Some children also present the condition at birth
• Although both genders may be affected, about 90% of the cases are seen in females
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Angioma Serpiginosum? (Predisposing Factors)

• A positive family history may be an important risk factor, since it is believed that Angioma Serpiginosum can be inherited
• A high incidence of the condition is noted in girls

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Angioma Serpiginosum? (Etiology)

• The exact cause of Angioma Serpiginosum is not known. In most cases, however, Angioma Serpiginosum occurs sporadically
• Some experts believe that the condition may be inherited in an autosomal dominant or X-linked manner
• It is reported that the X-linked form of the disorder is caused by a mutation in Xp11.3-Xq12 location of the X chromosome

What are the Signs and Symptoms of Angioma Serpiginosum?

The signs and symptoms of Angioma Serpiginosum may vary from one individual to another and may include:

• Presence of red skin lesions in the form of macules (flat discolored areas on skin)
• These usually develop on the buttocks and lower limbs, but any part of the body may be involved. The palms and soles are usually spared
• These lesions develop from tiny blood vessels on the skin surface that appear as red-purple dots (puncta); however, no bleeding is observed
• A collection of red-purple dots may form a serpiginous pattern (snake-like) on skin; the pattern may be linear, gyrate (ring-shape), or even irregular
• These red dots are noted along the lines of Blaschko. The Blaschko lines are defined as regions of the body where skin growth occurs in a systematic manner during fetal development. They are present, but not visible, all over the body
• The lesions develop over many months and years in young children. They are known to stop growing when the child reaches puberty, and further changes are not generally observed
• Thickening of the outer layer of skin (hyperkeratosis)
• The lesions are asymptomatic, with no pain, inflammation, or skin discoloration being noted
• An involvement of the mucosal surfaces is not noted

How is Angioma Serpiginosum Diagnosed?

Angioma Serpiginosum is diagnosed on the basis of the following information:

• Complete physical examination and thorough medical history evaluation
• Assessment of the presenting signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Angioma Serpiginosum?

The complications of Angioma Serpiginosum may include:

• Low self-esteem due to skin blemishes
• Cosmetic concerns
• Emotional stress
• Treatment resistance

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Angioma Serpiginosum Treated?

• In most cases, no treatment may be needed for Angioma Serpiginosum since it is typically a benign and asymptomatic condition
• If necessary, cosmetic masking or laser ablation may be considered

How can Angioma Serpiginosum be Prevented?

Angioma Serpiginosum may not be preventable, if it is an inherited condition.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

The risk factors or cause for the sporadic form of Angioma Serpiginosum are not known, and therefore, no guidelines or methods exist currently for its prevention.

What is the Prognosis of Angioma Serpiginosum? (Outcomes/Resolutions)

• The prognosis of Angioma Serpiginosum is typically good since it is a benign condition that may not require treatment in most cases
• However, the characteristic skin lesions may never disappear completely or disappear only partially in the affected individuals

Additional and Relevant Useful Information for Angioma Serpiginosum:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/