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

• KPAF (Keratosis Pilaris Atrophicans Faciei)

What is Keratosis Pilaris Atrophicans Faciei? (Definition/Background Information)

• Keratosis Pilaris Atrophicans Faciei (KPAF) is a genetic skin condition. It develops when hair follicles get plugged by dead skin. The skin lesions are mostly observed on the individual’s face
• Keratosis Pilaris Atrophicans Faciei 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
• The risk factors for Keratosis Pilaris Atrophicans Faciei include a positive family history, and other genetic disorders such as wooly hair syndrome, Rubinstein-Taybi syndrome, and Noonan syndrome
• A diagnosis of Keratosis Pilaris Atrophicans Faciei is made by clinical exam and various other diagnostic tools including dermoscopy and wood’s lamp examination
• The treatment of Keratosis Pilaris Atrophicans Faciei involves the use of topical moisturizers, steroids, and surgical procedures such as dermabrasion
• The prognosis of Keratosis Pilaris Atrophicans Faciei with adequate treatment is excellent since it is typically a harmless skin condition. However, the overall prognosis may be affected by the severity of any associated underlying genetic disorder

The other types of keratosis pilaris include:

• Atrophoderma Vermiculata (or Folliculitis Ulerythematosa Reticulata): This condition affects the skin resulting in pitted scarring of the cheek, which can cause cosmetic disfigurement
• Erythromelanosis Follicularis Faciei Et Colli: It usually affects the face and neck region
• Keratosis Follicularis Spinulosa Decalvans: It occurs on the scalp
• Ulerythema Ophryogenes: The eyebrows are typically affected

Who gets Keratosis Pilaris Atrophicans Faciei? (Age and Sex Distribution)

• Keratosis Pilaris Atrophicans Faciei is generally observed in children; the signs and symptoms usually begin during infancy
• The condition affects both males and females
• It is seen worldwide and all racial and ethnic groups may be affected

What are the Risk Factors for Keratosis Pilaris Atrophicans Faciei? (Predisposing Factors)

The risk factors for Keratosis Pilaris Atrophicans Faciei include the presence of a variety of genetic disorders including:

• A positive family history of KPAF
• Noonan syndrome: An inherited disorder that causes an abnormal development of various parts of the body
• Hereditary wooly hair syndrome: A condition that is marked by the presence of wooly (curly) hair
• Cardiofaciocutaneous (CFC) syndrome: A rare genetic condition affecting the heart, face, and skin
• Rubinstein-Taybi syndrome: A genetic disorder affecting physical and mental growth
• Cornelia de Lange syndrome: A genetic disorder characterised by many congenital defects
• Chromosome 18p deletion syndrome: A rare chromosomal condition causing developmental delays and mental growth deficiencies

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 Keratosis Pilaris Atrophicans Faciei?

Keratosis Pilaris Atrophicans Faciei (KPAF) is caused by the plugging of hair follicles by increased keratin debris (dead skin) on certain areas of the body.

• The condition is inherited in an autosomal dominant manner
• Some cases of KPAF may be sporadic and do not occur against a familial background
• There is no definitive proof that certain types of diet play a role in the development of this skin condition. Taking chocolates or oily food materials are not known to cause the condition
• KPAF 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

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 Keratosis Pilaris Atrophicans Faciei?

The signs and symptoms associated with Keratosis Pilaris Atrophicans Faciei may include:

• The presence of small elevated benign lesions that appear like a patch of tiny goose bumps, usually present on the face
• In the beginning, the lesions occur near the eyebrows. As they develop, they spread to other parts of the face including to the forehead and cheeks
• Occasionally, the skin lesions may be present in other parts of the body, such as on the arms and legs
• The onset of the signs and symptoms may take place during infancy. As the child moves into puberty, the signs and symptoms tend to get better
• Redness of skin around the lesions (erythema) is noted
• The skin feels like sandpaper, and occasionally they can itch
• The bumps can either take the surrounding skin color, or even be darker than the surrounding skin color (hyperpigmented)
• Additionally, the signs and symptoms of the underlying genetic disorder, if any present, may be observed

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 summer and worse during dry winter months, due to a lack of humidity
• Tanning of 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 without any seasonal variations (all year long).

How is Keratosis Pilaris Atrophicans Faciei Diagnosed?

The diagnosis of Keratosis Pilaris Atrophicans Faciei may involve:

• A complete evaluation of medical history along with a thorough examination of the skin lesions by a dermatologist
• The healthcare provider may also ask many questions related to the individual’s age, family medical history, current medications, cosmetics, body lotions used, other medical conditions, infections, 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
• 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 ordered
• Genetic testing to diagnose underlying associated disorder (if any present)
• 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 Keratosis Pilaris Atrophicans Faciei?

The complications associated with Keratosis Pilaris Atrophicans Faciei may include:

• Increased psychological stress due to the skin condition
• Cosmetic issues: The skin may be hypopigmented or hyperpigmented and can cause cosmetic issues
• The skin over the lesions may become thin (atrophic)
• Hair loss: With time, it can become permanent in the area of the skin lesions. It may be associated with scars (scarring alopecia)
• Scratching the skin lesions may result in scars on healing; the scars may be permanent
• The signs and symptoms may appear and disappear, even with treatment. The condition can recur

How is Keratosis Pilaris Atrophicans Faciei Treated?

There is no definite cure for Keratosis Pilaris Atrophicans Faciei (KPAF). The treatment options may include both medical and surgical treatment measures.

• The medical treatment for Keratosis Pilaris Atrophicans Faciei 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 (tretinoinand others) and mild corticosteroids
• The surgical care for Keratosis Pilaris Atrophicans Faciei may include:
  • Dermabrasion: It is a surgical procedure to treat KPAF causing cosmetic issues
  • Laser therapy: Despite all the above treatment measures, if no improvement is noted, then laser therapy may be employed
• Additionally, suitable treatment of the underlying and associated disorder may be necessary

Note:

• One has to be patient for the signs and symptoms to get better, since it may take months before showing any improvement. Once the 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 Keratosis Pilaris Atrophicans Faciei:

• Keeping the skin moist can help in reducing the signs and symptoms caused by KPAF
• If any of the prescribed treatment causes new stinging or burning symptoms, then it is important to stop using them immediately and inform the physician’s office
• Taking care of oneself, being clean and hygienic (especially face and hands), 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 the time spent in water
• Completely avoid scratching the affected areas

How can Keratosis Pilaris Atrophicans Faciei be Prevented?

Currently, there are no specific methods or guidelines to prevent Keratosis Pilaris Atrophicans Faciei. 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 Keratosis Pilaris Atrophicans Faciei? (Outcomes/Resolutions)

• The prognosis of Keratosis Pilaris Atrophicans Faciei (KPAF) is excellent in a majority of cases, since it is a benign/harmless condition. However, the overall prognosis may depend upon the severity of the underlying condition, if any present
• Severe cases of KPAF may lead to cosmetic issues, which can be addressed through appropriate treatment measures. In some individuals, KPAF signs and symptoms can get better as one ages
• Regular follow up visits with the healthcare providers are important

Additional and Relevant Useful Information for Keratosis Pilaris Atrophicans Faciei:

• 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 Keratosis Pilaris Atrophicans Faciei. However, it helps to be clean and hygienic, which will help the condition from getting worse