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

• EFF (Erythromelanosis Follicularis Faciei Et Colli)
• EFFC (Erythromelanosis Follicularis Faciei Et Colli)
• Erythromelanosis

What is Erythromelanosis Follicularis Faciei Et Colli? (Definition/Background Information)

• Erythromelanosis Follicularis Faciei Et Colli (EFFC) is an unusual skin disorder that is marked by a triad of conditions including increased skin pigmentation, plugging of hair follicles, and redness of the face and neck areas
• Erythromelanosis Follicularis Faciei Et Colli is a rare subtype of keratosis pilaris (or KP, a common skin condition). Keratosis pilaris results in the formation of small bumps on the skin, usually on the arms and legs, and sometimes on the lower cheeks
• The cause of the condition is unknown and no specific risk factors for Erythromelanosis Follicularis Faciei Et Colli are noted. The majority of the cases appears to occur sporadically
• A diagnosis of Erythromelanosis Follicularis Faciei Et Colli is made by clinical exam and various other diagnostic tools including dermoscopy and Wood’s lamp examination
• The treatment of Erythromelanosis Follicularis Faciei Et Colli includes the use of topical moisturizers, steroids, and surgical procedures such as dermabrasion
• The prognosis of Erythromelanosis Follicularis Faciei Et Colli with adequate treatment is excellent, since generally it is a harmless condition

There are several types of keratosis pilaris, and these include:

• Atrophoderma Vermiculata (or Folliculitis Ulerythematosa Reticulata): This condition affects the skin resulting in pitted scarring of the cheek, which can cause cosmetic disfigurement
• Keratosis Follicularis Spinulosa Decalvans: It occurs on the scalp and other sites
• Keratosis Pilaris Atrophicans Faciei: The condition usually involves the face
• Ulerythema Ophryogenes: The eyebrows are typically affected in this subtype

Who gets Erythromelanosis Follicularis Faciei Et Colli? (Age and Sex Distribution)

• Erythromelanosis Follicularis Faciei Et Colli is a rare condition generally observed in teenagers and young adults
• The condition affects both males and females, though males are predominantly affected
• It is seen worldwide and all racial and ethnic groups may be affected
• EFFC is more typically associated with Asian men; clusters of cases have been reported in India

What are the Risk Factors for Erythromelanosis Follicularis Faciei Et Colli? (Predisposing Factors)

• Presently, there are no specific risk factors identified for Erythromelanosis Follicularis Faciei Et Colli
• However, a family history of the condition may be a contributing factor

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 Erythromelanosis Follicularis Faciei Et Colli?

Erythromelanosis Follicularis Faciei Et Colli (EFFC) is caused by the plugging of hair follicles by increased keratin debris (dead skin) on certain areas of the body.

• A majority of the cases are sporadic, although some researchers believe that it could be an autosomal dominant (inherited) condition and a family history may play a role in its development
• There is no definitive proof that certain types of diet play a role in the formation of EFFC. Consuming chocolates or oily food items are not known to cause the condition
• EFFC is non-contagious and it does not spread from one individual to another. In other words, one cannot contract the condition by interacting closely with the affected individuals. It is not a sexually-transmitted disease too

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly functioning gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Erythromelanosis Follicularis Faciei Et Colli?

The signs and symptoms associated with Erythromelanosis Follicularis Faciei Et Colli (EFFC) may include:

• EFFC is marked by the following three characteristic features:
  • Increased pigmentation (hyperpigmentation)
  • Hair follicle plugging
  • Redness of face and neck (facial erythema)
• The presence of small elevated benign lesions that appear like a patch of tiny goose bumps, usually present on the face
• The condition is symmetrical and may affect both sides of the face
• The lesions are present on the cheeks, temples, and extending on the sides of the neck
• It can be associated with keratosis pilaris elsewhere on the body
• Increased pigmentation is seen on the affected area; the lesions are red-brown in color
• The skin feels like sandpaper, and occasionally it can be itchy
• The bumps can either take the surrounding skin color or be even darker (hyperpigmented)

Aggravation of the condition may occur under the following circumstances:

• In dry, low-humid conditions, the symptoms may aggravate because of skin dryness. It may get better during the summer months and worse during dry winter months, due to a lack of humidity
• Tanning of the skin through sun exposure
• Shaving or waxing of skin
• Dry sauna can also aggravate this skin condition

Note: Individuals, nearer to the tropics, may see the condition remain the same (all year long) without any seasonal variations.

How is Erythromelanosis Follicularis Faciei Et Colli Diagnosed?

The diagnosis of Erythromelanosis Follicularis Faciei Et Colli may involve:

• A complete evaluation of medical history along with a thorough examination of the skin lesions by a dermatologist
• The healthcare provider may ask several questions related to the individual’s age, family medical history, current medications, cosmetics, body lotions used, presence of other medical conditions/infections (if any), etc.
• 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
• Lab tests such as:
  • Complete blood count (CBC) test
  • Examination of peripheral blood smear under a microscope by a pathologist
  • If secondary infections develop, then a culture test may be done
• In very rare cases, a skin biopsy may be performed: 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

Note: If itching is a prominent symptom, then other conditions, such as fungal infections, should be ruled out.

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 Erythromelanosis Follicularis Faciei Et Colli?

Complications associated with Erythromelanosis Follicularis Faciei Et Colli include:

• Increased psychological stress due to the skin condition
• Cosmetic issues: The skin may be hypopigmented or hyperpigmented and cause cosmetic issues
• The skin over the lesions may become thin (atrophic)
• Hair loss: With time, hair loss can become permanent in the area of the skin lesions
• Scratching the skin lesions may result in scars on healing; the scars may be permanent

How is Erythromelanosis Follicularis Faciei Et Colli Treated?

There is no definite cure for Erythromelanosis Follicularis Faciei Et Colli (EFFC). The treatment options may include both medical and surgical treatment measures.

• The conservative treatments for Erythromelanosis Follicularis Faciei Et Colli may include:
  • Avoiding hot dry environment
  • Use of moisturizers with urea or lactic acid
  • Topical medicated creams may be administered to remove dead skin (exfoliation therapy). The common medications used include lactic acid, vitamin D, and salicylic acid
  • Use of topical retinoids and steroids
  • For severe cases, oral isotretinoin can be used for short courses
• The surgical care for Erythromelanosis Follicularis Faciei Et Colli may include:
  • Dermabrasion: It is a surgical procedure to treat EFFC causing cosmetic issues
  • Laser therapy: Despite all the above treatment measures, if no improvement is noted, then laser therapy may be employed

Note:

• Isotretinoin should NOT be used on pregnant women, or on women who are planning to become pregnant. This is very important because isotretinoin can cause fetal abnormalities and malformations, during the growth of the fetus in the mother’s womb, termed teratogenic defects of isotretinoin. Hence, a pregnancy test is usually performed in women of child-bearing age before starting treatment with isotretinoin
• One has to be patient for the signs and symptoms to get better, since it may take months before showing any improvement. Once treatment has started, one has to be consistent in following the prescriptions and taking the medications continuously and regularly. Skipping the treatment regimen will not help improve the condition

The following tips are helpful for Erythromelanosis Follicularis Faciei Et Colli:

• Keeping the skin moist can help in reducing the signs and symptoms caused by EFFC
• If any of the prescribed treatment causes new stinging or burning symptoms, then it is important to stop using them (the medications) immediately and inform the physician’s office
• Taking care of oneself, being clean and hygienic (especially keeping the face and hands clean), avoiding oily make-ups and creams, drinking lots of water, etc. are all simple practices that may help ensure a faster recovery from the condition
• Avoid excessive washing and scrubbing of the skin; also limit time spent in water
• Completely avoid scratching the affected skin areas

How can Erythromelanosis Follicularis Faciei Et Colli be Prevented?

Currently, there are no specific methods or guidelines to prevent Erythromelanosis Follicularis Faciei Et Colli. However, in order to prevent aggravation of the condition, the following steps may be considered:

• Use of home humidifiers, in case of dry indoor air
• Use moisturizing creams regularly to prevent dry skin
• Use mild bathing soaps to not aggravate the condition
• Avoid excessive, long duration baths using hot water
• While showering do not use rough scrubbers, it can make the condition worse
• Avoid tanning of the skin
• Avoid shaving or skin waxing

What is the Prognosis of Erythromelanosis Follicularis Faciei Et Colli? (Outcomes/Resolutions)

• The prognosis of Erythromelanosis Follicularis Faciei Et Colli (EFFC) is excellent in a majority of cases, since it is a benign/harmless condition
• Severe cases of EFFC may lead to cosmetic issues, which can be addressed through appropriate treatment measures. While, in some individuals, the signs and symptoms are known to get better as one ages
• Regular follow up visits with the healthcare provider is important

Additional and Relevant Useful Information for Erythromelanosis Follicularis Faciei Et Colli:

• 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 for Erythromelanosis Follicularis Faciei Et Colli. However, it helps to be clean and hygienic, which will help the condition from getting worse