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

• Elasotsis Intrapapillare Perforans Verruciformis
• Keratosis Follicularis et Parafollicularis Serpiginosa
• Lutz-Miescher Disease

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

• Elastosis Perforans Serpiginosa (EPS) is a rare skin disorder that can manifest during adulthood. In this abnormal condition, elastic fibers come out of (extrude from) the epidermis of skin
• There are three types of Elastosis Perforans Serpiginosa:
  • Idiopathic EPS: The cause of this form is unknown
  • Reactive EPS: It is known to occur with connective tissue disorders such as Marfan’s syndrome and Ehlers-Danlos syndrome. Individuals with Down syndrome can also get the reactive form of Elastosis Perforans Serpiginosa
  • Drug-Induced EPS: It occurs as a side effect of certain medications, such as pencillamine
• Elastosis Perforans Serpiginosa is a chronic disorder that is characterized by the presence of multiple tiny papules on the neck or face in a winding pattern (snake-like). The skin lesions are usually itchy
• The treatment for Elastosis Perforans Serpiginosa may involve vitamin A therapy, antihistamine, and moisturizing creams. However, there is no cure for both the idiopathic and reactive form of EPS. Stopping or discontinuing the use of the causative medication (or topical cream) may decrease the progression of or cure the drug-induced form of EPS
• The prognosis of Elastosis Perforans Serpiginosa is good with treatment, but it depends upon the severity of the condition. Moreover, the symptoms can be longstanding; it may appear, subside, and reappear over a long time

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

• 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. Some studies show that adults in the 20-50 year age group are affected the most
• It can affect both males and females, though there is a male dominance (male-female ratio is 4:1)
• The condition can occur worldwide; individuals of all racial and ethnic background may be affected

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

The exact cause of Elastosis Perforans Serpiginosa (EPS) development is generally unknown.

• The risk factors for the idiopathic form is unknown
• The risk factors for the reactive form include connective tissue disorders (such as Marfan’s syndrome and Ehlers-Danlos syndrome), genetic disorders (such as Down syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum), and scleroderma
• The administration of certain medications, such as pencillamine, may induce the drug-induced form of Elastosis Perforans Serpiginosa

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 Elastosis Perforans Serpiginosa? (Etiology)

The exact cause of Elastosis Perforans Serpiginosa development is unknown.

• The idiopathic type of EPS has no established cause
• The reactive type of EPS may be influenced by the presence of certain connective tissue disorders and genetic disorders
• The drug-induced type of EPS occurs as an adverse reaction of the body to the use of certain medications/topical applications

What are the Signs and Symptoms of Elastosis Perforans Serpiginosa?

The signs and symptoms of Elastosis Perforans Serpiginosa 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
• In EPS, elastic fibers 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 also be affected
• 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 over many years).

How is Elastosis Perforans Serpiginosa Diagnosed?

The following are the diagnostic methods that may be used for Elastosis Perforans Serpiginosa (EPS):

• 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 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
• In evaluating a biopsy of possible EPS, the pathologist has the laboratory staff prepare slides which specifically stain elastin fibers to aid in 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 Elastosis Perforans Serpiginosa?

The complications due to Elastosis Perforans Serpiginosa (EPS) may include:

• Elastosis Perforans Serpiginosa 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 and emotional and psychological stress
• Bacterial and fungal infections: EPS can cause continuous itching and scaling of the skin, which creates skin moistness, providing a suitable environment for bacteria and fungi to grow and thrive

How is Elastosis Perforans Serpiginosa Treated?

Currently, there is no cure for the idiopathic and reactive form of Elastosis Perforans Serpiginosa (EPS). Discontinuing the medication/drug responsible for the side effect can result in a cure or improvement of the condition, in case of Drug-Induced EPS.

The treatment strategies for Elastosis Perforans Serpiginosa may include:

• Retinoid therapy, using either vitamin A, or medications patterned after vitamin A called retinoids. These may be used topically or given orally
• 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
• Undertaking proper treatment and management of the underlying condition/disorder (if any)

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

A few self-care tips and home remedies for Elastosis Perforans Serpiginosa 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 caused by EPS

How can Elastosis Perforans Serpiginosa be Prevented?

• Currently, there are no specific methods or guidelines to prevent Elastosis Perforans Serpiginosa (EPS)
• Drugs that cause the drug-induced form of EPS may be discontinued or alternative medications used
• Undertaking proper treatment of associated disorders can be ensured to lower one’s risk for EPS

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

• The prognosis of Elastosis Perforans Serpiginosa (EPS) depends upon the severity of the condition; mild cases (idiopathic and reactive forms) have better prognosis than severe ones
• It is also dependent upon the underlying health condition, if any, associated with it. Some cases may be chronic and last for many years
• The drug-induced form of EPS typically has good prognosis once usage of the offending drug is removed or stopped
• Regular follow up visits with the healthcare providers are important

Additional and Relevant Useful Information for 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