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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.


Keratosis pilaris atrophicans faciei — codes and concepts
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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 particularly in the eyebrows [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’, and 'ophryo' refers to the eyebrow.

Keratosis pilaris atrophicans faciei

See more images of keratosis pilaris.

Who gets keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei usually occurs sporadically; however, autosomal dominant and autosomal recessive inheritance have been described [1,2,3]. It first appears in infancy, and is mainly diagnosed in children and adolescents.

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 facial keratotic papules and erythematous keratotic follicular papules involving the lateral third of the eyebrows.
  • As the condition progresses, atrophy and loss of eyebrow hair occur.
  • Other areas of the scalp and face can be involved including the forehead, temples, and cheeks [1,3].
  • There is usually also widespread keratosis pilaris on limbs and trunk [3].

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 a widened infundibulum, plugged hair follicle, loss of hair follicle, superficial perivascular lymphocytic infiltrate, and dermal fibrosis [1,3].

What is the differential diagnosis for keratosis pilaris atrophicans faciei?

Keratosis pilaris atrophicans faciei is on a spectrum with other follicular keratotic disorders, and is usually regarded as one form of keratosis pilaris atrophicans, with overlapping features commonly seen [3,5].

Keratosis pilaris rubra faciei

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

Erythromelanosis follicularis faciei et colli

Erythromelanosis follicularis faciei et colli is a variant of keratosis pilaris characterised by well-defined patches of erythema, hyperpigmentation, and follicular papules on the cheeks and neck. Most cases have been reported in Asian men [5].

Atrophoderma vermiculatum

Atrophoderma vermiculatum is a variant of keratosis pilaris atrophicans with prominent follicular atrophy resulting in a honeycombed (or worm-eaten) pattern on the cheeks developing in childhood [3,5].

Atrophoderma vermiculatum

Keratosis follicularis spinulosa decalvans

Keratosis follicularis spinulosa decalvans is a severe form of keratosis pilaris atrophicans with widespread follicular papules, scarring hair loss, keratoderma and keratitis [3,5].

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?

There is no cure 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.

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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(1):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(8):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(2):83-7. PubMed
  5. Judge MR, McLean WHI, Munro CS. Disorders of keratinization. In: Burns T, Breathnach S, Cox N, Griffiths C (eds). Rook's Textbook of Dermatology, 7th edn, Oxford: Blackwell Publishing, 2004.

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