DermNet provides Google Translate, a free machine translation service. Note that this may not provide an exact translation in all languages

Keratosis pilaris atrophicans faciei

Author: Olivia Egan, Medical Student, University of Sydney, Sydney, NSW, Australia. DermNet NZ Technical Editor: Elaine Mary Luther, Medical Student, Ross University School of Medicine, Barbados. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. February 2020.


What is keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei is an uncommon form of keratosis pilaris with scar-like follicular depressions and loss of hair primarily in the eyebrow area [1]. This results in atrophy and permanent loss of hair in the affected areas.

Keratosis pilaris is also called ulerythema ophryogenes. Ulerythema means ‘scar with redness’.

Keratosis pilaris atrophicans faciei

See more images of keratosis pilaris.

Who gets keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei mostly occurs sporadically; however, autosomal dominant and autosomal recessive inheritance have been described [1,2]. It is mainly diagnosed in children and adolescents, but can persist into adulthood.

What causes keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei is caused by abnormal keratinisation of the follicular infundibulum. Scale fills the follicle and obstructs the hair shaft resulting in inflammation. Over time, chronic inflammation results in atrophy and alopecia [1].

Some cases of keratosis pilaris are due to an autosomal recessive mutation in the desmoglein 4 (DSG4) gene [3]. The rare association of keratosis pilaris atrophicans faciei with some congenital syndromes also suggests that chromosome arm 18p might contain genes for follicular keratinisation and be involved in disease pathogenesis [4].

What are the clinical features of keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei is characterised by facial inflammatory keratotic papules.

  • Onset is usually a few months after birth with signs of erythema and keratotic follicular papules involving the lateral third of the eyebrow.
  • As the condition progresses, atrophy and loss of eyebrow hair occur.
  • Other areas of the face that can be involved include the forehead, temples, and cheeks [1].

Keratosis pilaris atrophicans faciei

Associated syndromes

Keratosis pilaris atrophicans faciei is occasionally associated with Noonan syndrome, Rubinstein-Taybi syndrome, Cornelia de Lange syndrome, and cardiofaciocutaneous syndrome [4].

How is keratosis pilaris atrophicans faciei diagnosed?

Keratosis pilaris atrophicans faciei is diagnosed clinically.

If a skin biopsy is performed, nonspecific histopathologic features include plugged hair follicle, loss of hair follicle, superficial perivascular lymphocytic infiltrate, and dermal fibrosis [1].

What is the differential diagnosis for keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei is on a spectrum with other follicular keratotic disorders.

Keratosis pilaris rubra faciei

Keratosis pilaris rubra faciei is a variant of keratosis pilaris in which redness is emphasised. It is characterised by perifollicular erythema on the forehead, cheeks, and neck without any atrophy or hair loss.

Erythromelanosis follicularis faciei et colli

Erythromelanosis follicularis faciei et colli is a variant of keratosis pilaris in which pigmentation is present. It is characterised by erythema, hyperpigmentation, and follicular papules on the cheeks, preauricular skin, and neck, and affects children and adults with darker skin.

Atrophoderma vermiculatum

Atrophoderma vermiculatum is a variant in which follicular atrophy is prominent, resulting in a honeycombed (or worm-eaten) pattern on the cheeks.

Atrophoderma vermiculatum

Keratosis follicularis spinulosa decalvans

Keratosis follicularis spinulosa decalvans is a similar disorder affecting the scalp.

Frontal fibrosing alopecia

Frontal fibrosing alopecia is a type of follicular lichen planus or lichen planopilaris in which there are facial follicular papules, loss of the lateral eyebrows, and receding frontal hairline due to scarring alopecia. It mainly affects postmenopausal women.

What is the treatment for keratosis pilaris atrophicans faciei?

No cure is available for keratosis pilaris atrophicans faciei. The following can be useful to minimise the follicular scale.

Pulse dye laser or intense pulsed light (IPL) can reduce erythema temporarily but it tends to recur. Sun exposure tends to worsen redness, so sun protection is recommended.

What is the outcome for keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei usually becomes less noticeable after puberty. However, atrophy with hair loss is permanent.

See smartphone apps to check your skin.
[Sponsored content]

 

Related information

 

References

  1. Landis MN, Fowler J, Corona R. Keratosis pilaris atrophicans. In: Post TW (ed). UpToDate [internet]. Waltham MA: UpToDate Inc; 2020. Available from: www.uptodate.com/contents/keratosis-pilaris-atrophicans [cited 3 Feb 2020]
  2. Callaway SR, Lesher JL Jr. Keratosis pilaris atrophicans: case series and review. Pediatr Dermatol 2004; 21: 14–17. doi:10.1111/j.0736 8046.2004.21103.x. PubMed
  3. Cohen-Barak E, Danial-Farran N, Hammad H, et al. Desmoglein 4 mutation underlies autosomal recessive keratosis pilaris atrophicans. Acta Derm Venereol 2018; 98: 809–10. doi:10.2340/00015555-2976. PubMed
  4. Morton CM, Bhate C, Janniger CK, Schwartz RA. Ulerythema ophryogenes: updates and insights. Cutis 2014; 93: 83–7. PubMed

On DermNet NZ

Other websites

Books about skin diseases