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

• Reactive EPS
• Reactive Perforating Elastosis

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

• Elastosis Perforans Serpiginosa (EPS) is a rare skin disorder usually seen during adulthood. In this abnormal condition, elastic fibers come out of (extrude from) the epidermis of skin. There are three types of EPS: Idiopathic, Reactive, and Drug-Induced form
• In Reactive Elastosis Perforans Serpiginosa, the cause of the condition remains unknown. However, it is known to occur in association with many connective tissue disorders and genetic disorders
• The condition is characterized by the presence of multiple tiny papules on the neck or face in a winding pattern. The skin lesions are usually itchy, which can result in infections on scratching
• The treatment for Reactive Elastosis Perforans Serpiginosa may involve vitamin A therapy, antihistamine, and moisturizing creams. However, there is no cure for the reactive form of EPS
• In general, the prognosis of Reactive Elastosis Perforans Serpiginosa is good with treatment, but it also depends upon the severity of the condition and the associated/underlying disorder. The condition can be chronic and longstanding; the symptoms may come and go over a long period of time

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

• Reactive 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 predominance is observed
• The condition can occur worldwide; individuals of all racial and ethnic background may be affected

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

The risk factors for Reactive Elastosis Perforans Serpiginosa include the following conditions:

• Connective tissue disorders such as Marfan’s syndrome and Ehlers-Danlos syndrome
• Genetic disorders such as Down syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum
• Skin conditions such as scleroderma

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 Reactive Elastosis Perforans Serpiginosa? (Etiology)

• The reactive type of Elastosis Perforans Serpiginosa has no established cause
• However, the contributory factors for the condition include certain genetic syndromes and connective tissue disorders

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

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

• The presence of multiple 2-6 mm sized skin papules in a curvilinear snake-like pattern; there may be a central keratotic plug observed among the collection of papules
• In this condition, elastic fibers extrude from the skin surface of the lesions. This is detected microscopically
• 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 (usually years).

How is Reactive Elastosis Perforans Serpiginosa Diagnosed?

The following are the diagnostic methods that may be used for Reactive 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 Reactive EPS may be arrived at clinically by analyzing the signs and symptoms and medical history. 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
• Tests and procedures to identify underlying disorders, if any

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 Reactive Elastosis Perforans Serpiginosa?

The complications due to Reactive Elastosis Perforans Serpiginosa may include:

• It can be a longstanding and chronic condition; often the skin symptoms ‘comes and goes’ over a period of many years
• It can lead to self-image issues and 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
• Complications of underlying disorders

How is Reactive Elastosis Perforans Serpiginosa Treated?

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

• Topical retinoid cream therapy (tretinoin, tazarotene)
• 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
• Laser ablative treatment
• Reassurance may be provided in case of emotional stress
• Undertaking treatment for underlying disorders and syndromes, if any present

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 Reactive Elastosis Perforans Serpiginosa be Prevented?

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

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

• The prognosis of Reactive Elastosis Perforans Serpiginosa depends upon the severity of the condition; mild cases have better prognosis than severe ones
• It is also dependent upon the underlying health condition or disorder, if any, associated with it
• Some cases may be chronic and last for many years
• Regular follow up visits with the healthcare providers are important and encouraged

Additional and Relevant Useful Information for Reactive 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