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Keratosis Follicularis Spinulosa Decalvans

Last updated Oct. 22, 2018

Approved by: Maulik P. Purohit MD, MPH

Keratosis Follicularis Spinulosa Decalvans is a skin disorder that is believed to be an X-linked genetic condition, since a majority of the individuals affected are males. It develops when hair follicles get plugged by dead skin.


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

  • KFSD (Keratosis Follicularis Spinulosa Decalvans)

What is Keratosis Follicularis Spinulosa Decalvans? (Definition/ Background Information)

  • Keratosis Follicularis Spinulosa Decalvans (KFSD) is a skin disorder that is believed to be an X-linked genetic condition, since a majority of the individuals affected are males. It develops when hair follicles get plugged by dead skin. The condition leads to hair loss on the scalp, eyebrows, and eyelashes
  • Keratosis Follicularis Spinulosa Decalvans 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
  • Specific risk factors for Keratosis Follicularis Spinulosa Decalvans are unknown, but it is known to occur in association with conditions including palmoplantar keratoderma and tufted hair folliculitis
  • A diagnosis of Keratosis Follicularis Spinulosa Decalvans is made by clinical exam and various other diagnostic tools including dermoscopy and wood’s lamp examination
  • The treatment of Keratosis Follicularis Spinulosa Decalvans includes the use of topical moisturizers, steroids, and surgical procedures such as dermabrasion
  • The prognosis of Keratosis Follicularis Spinulosa Decalvans with adequate treatment is good, since it is a benign condition. However, the overall prognosis may depend upon the severity of the symptoms, the response to treatment, and severity of the associated condition (if any)

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 Pilaris Atrophicans Faciei: The condition usually affects the face
  • Ulerythema Ophryogenes: The eyebrows are typically affected

Who gets Keratosis Follicularis Spinulosa Decalvans? (Age and Sex Distribution)

  • Keratosis Follicularis Spinulosa Decalvans is generally observed in children and teenagers. However, individuals of a wider age range may be affected
  • The condition affects both males and females, although males are predominantly affected
  • It is seen worldwide and all racial and ethnic groups may be affected

What are the Risk Factors for Keratosis Follicularis Spinulosa Decalvans? (Predisposing Factors)

The risk factors for Keratosis Follicularis Spinulosa Decalvans include the following:

  • A positive family history of KFSD
  • It may be associated with the following conditions:
    • Palmoplantar keratoderma, resulting in rough skin (lesions) on the soles/palms
    • Acne keloidalis nuchae, a skin condition forming papules and pustules
    • Tufted hair folliculitis, a rare disorder that leads to scarring alopecia

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 Follicularis Spinulosa Decalvans?

Keratosis Follicularis Spinulosa Decalvans is caused by the plugging of hair follicles by increased keratin debris (dead skin) on certain areas of the body.

  • In individuals with a family history of KFSD, the condition may follow an X-linked inheritance pattern. Occasionally, it may be inherited in an autosomal dominant manner. The pattern of inheritance is dependent upon the involved gene, or the gene that is mutated
  • The condition may also occur sporadically, when no familial background is noted
  • 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
  • KFSD 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 Follicularis Spinulosa Decalvans?

The signs and symptoms associated with Keratosis Follicularis Spinulosa Decalvans may include:

  • The presence of small elevated benign lesions that appear like a patch of tiny goose bumps
  • The signs and symptoms begin during early childhood
  • Extensive involvement of the face, scalp, arms, and legs are noted
  • The skin feels like sandpaper, and occasionally they can itch
  • There can be redness of skin around the rashes or bumps (erythema)
  • The bumps can either take the surrounding skin color, or even be darker than the surrounding skin color (hyperpigmented)
  • It leads to hair loss on the scalp, eyelashes, and eyebrows
  • The hair loss is usually associated with scarring (a condition known as cicatricial alopecia)

Other signs and symptoms associated with KFSD may include:

  • Sensitivity to light (photophobia)
  • Vision abnormalities due to corneal dystrophy
  • Blepharitis, or inflammation of the eyelid
  • Individuals may have signs and symptoms related to the associated underlying disorder

Aggravation of the condition may occur under the following circumstances:

  • In children, the signs and symptoms may become worse during puberty
  • 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 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 without any seasonal variations (all year long).

How is Keratosis Follicularis Spinulosa Decalvans Diagnosed?

The diagnosis of Keratosis Follicularis Spinulosa Decalvans 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.
  • Eye examination by a healthcare specialist, if necessary, along with the following tests, depending on the signs and symptoms:
    • Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye
    • Visual acuity test using a special and standardized test chart (Snellen chart)
    • Slit-lamp examination: Examination of the eye structure by dilating the pupils using a special instrument (a slit-lamp)
  • 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)
  • 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 Keratosis Follicularis Spinulosa Decalvans?

Complications associated with Keratosis Follicularis Spinulosa Decalvans 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, it can become permanent in the affected areas (scalp, eyelashes, and eyebrows)
  • Scratching the skin lesions may result in scars on healing
  • Severe vision loss
  • The signs and symptoms may appear and disappear, even with treatment. The condition can recur

How is Keratosis Follicularis Spinulosa Decalvans Treated?

There is no definite cure for Keratosis Follicularis Spinulosa Decalvans after onset of the signs and symptoms. The treatment options for KFSD may include both medical and surgical treatment measures.

  • The medical treatment for Keratosis Follicularis Spinulosa Decalvans 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 (isotretinoin) and steroids
    • Treatment for eye-related signs and symptoms
  • The surgical care for Keratosis Follicularis Spinulosa Decalvans may include:
    • Dermabrasion: It is a surgical procedure to treat KP 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:

  • 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 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 Follicularis Spinulosa Decalvans:

  • Keeping the skin moist can help in reducing the signs and symptoms caused by KFSD
  • 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 time spent in water
  • Completely avoid scratching the affected areas

How can Keratosis Follicularis Spinulosa Decalvans be Prevented?

Currently, there are no specific methods or guidelines to prevent Keratosis Follicularis Spinulosa Decalvans. 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
  • Avoid bright sunlight and UV light

What is the Prognosis of Keratosis Follicularis Spinulosa Decalvans? (Outcomes/Resolutions)

  • The prognosis of Keratosis Follicularis Spinulosa Decalvans may depend on a set of several factors including:
    • Severity of the signs and symptoms
    • Response to therapy
    • Association with any underlying disorder
  • However, it is important to note that KFSD is a benign skin condition and the prognosis is good in a majority of cases with appropriate treatment
  • Regular follow up visits with the healthcare providers are important

Additional and Relevant Useful Information for Keratosis Follicularis Spinulosa Decalvans:

  • There are overlapping features between Keratosis Follicularis Spinulosa Decalvans and a syndrome called ichthyosis follicularis atrichia photophobia syndrome (IFAP syndrome), which is an X-linked inherited condition
  • 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 KFSD. However, it helps to be clean and hygienic, which will help prevent the condition from getting worse

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Oct. 2, 2016
Last updated: Oct. 22, 2018