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

• Idiopathic EPS

What is Idiopathic Elastosis Perforans Serpiginosa? (Definition/Background Information)

• Elastosis Perforans Serpiginosa (EPS) is a rare skin disorder seen during adulthood. In this abnormal condition, elastic fibers come out of (extrude from) the epidermis of skin. There are 3 types of EPS: Idiopathic, Reactive, and Drug-Induced form
• In Idiopathic Elastosis Perforans Serpiginosa, the cause and risk factors for the condition remain unknown. EPS is characterized by the presence of multiple tiny papules on the neck or face in a meandering, winding pattern. The skin lesions are usually itchy
• The treatment for Idiopathic Elastosis Perforans Serpiginosa may involve oral vitamin A therapy, antihistamines, and moisturizing creams. However, there is no cure for the idiopathic form of EPS
• In general, the prognosis of Idiopathic Elastosis Perforans Serpiginosa is good with treatment, but it also depends upon the severity of the condition. Moreover, the condition can be chronic and longstanding; the symptoms may come and go over a long period of time

Who gets Idiopathic Elastosis Perforans Serpiginosa? (Age and Sex Distribution)

• Idiopathic Elastosis Perforans Serpiginosa is a rare disorder that generally affects young adults in their 20s. However, both children and elderly adults may also be affected
• Even though both males and females are affected, a male dominance is observed
• The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Idiopathic Elastosis Perforans Serpiginosa? (Predisposing Factors)

• The risk factors for Idiopathic Elastosis Perforans Serpiginosa is unknown

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Idiopathic Elastosis Perforans Serpiginosa? (Etiology)

The idiopathic type of Elastosis Perforans Serpiginosa has no established cause.

What are the Signs and Symptoms of Idiopathic Elastosis Perforans Serpiginosa?

The signs and symptoms of Idiopathic Elastosis Perforans Serpiginosa may include:

• The presence of multiple, 2-6 mm sized skin papules in a snake-like pattern; there may be a central keratotic plug observed among the collection of papules
• Elastic fibers can be observed to extrude from the skin surface of the lesions
• The lesions are usually itchy
• The affected area includes the head and neck region, especially the neck and face. The arms and legs may be affected too
• The condition may be bilateral i.e., both sides of the body are affected

The symptoms appear and disappear (come and go) over a long period of time (usually years).

How is Idiopathic Elastosis Perforans Serpiginosa Diagnosed?

The following are the diagnostic methods that may be used for Idiopathic Elastosis Perforans Serpiginosa:

• A thorough physical examination and a complete medical history are very crucial
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• A diagnosis of Idiopathic EPS may be arrived at clinically by analyzing the signs and symptoms. A skin biopsy is usually not needed
• Skin biopsy: A skin 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

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 Idiopathic Elastosis Perforans Serpiginosa?

The complications due to Idiopathic Elastosis Perforans Serpiginosa may include:

• It can be a longstanding and chronic condition; often the skin symptoms ‘come and go’ over a period of many years
• It can lead to self-image issues, emotional and psychological stress
• Bacterial and fungal infections: Due to continuous itching and scaling of the skin, it can result in prolonged moist skin, which provides a suitable environment for bacteria and fungi to grow and thrive

How is Idiopathic Elastosis Perforans Serpiginosa Treated?

Presently, Idiopathic Elastosis Perforans Serpiginosa has no cure. The treatment strategies that may be adopted for this type of skin disorder may include:

• Vitamin A therapy
• Phototherapy - the use of light to treat the skin condition
• Use of moisturizing creams
• Antihistamines to help with itching
• For mild skin conditions, self-care measures are recommended, such as washing with mild (antibacterial) soap and applying warm compress
• Reassurance may be provided in case of emotional stress

Follow-up care with regular screening and checkups are important.

A few self-care tips and home remedies for EPS may include:

• Completely avoid scratching the affected areas
• A comfortable, cool bath may help soothe the skin; but, avoid excessive washing and scrubbing of the skin
• Wear smooth cotton clothes
• Use only mild perfumes, soaps, and detergents
• Keeping the skin moist can help in reducing the signs and symptoms

How can Idiopathic Elastosis Perforans Serpiginosa be Prevented?

Currently, there are no specific methods or guidelines to prevent Idiopathic Elastosis Perforans Serpiginosa.

What is the Prognosis of Idiopathic Elastosis Perforans Serpiginosa? (Outcomes/Resolutions)

• The prognosis of Idiopathic Elastosis Perforans Serpiginosa depends upon the severity of the condition; mild cases have better prognosis than severe ones
• Some cases may be chronic and last for many years
• Regular follow up visits with the healthcare providers are important

Additional and Relevant Useful Information for Idiopathic Elastosis Perforans Serpiginosa:

• There is no evidence to prove that oily foods and chocolate-based products have an influence on Elastosis Perforans Serpiginosa
• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor. However, it helps to be clean and hygienic, which will help the condition from getting worse