Pediatric Keratosis Pilaris

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Keratosis pilaris is a very common, benign, heritable disorder of keratinized hair follicles. [1] It is characterized by grouped, horny, keratotic, follicular papules, predominantly located over the extensor surfaces of the proximal extremities, most commonly the posterolateral upper arms and anterior thighs. Though the papules are typically asymptomatic, affected individuals may be bothered by the cosmetic appearance. There can be significant individual variation in the prominence and severity of keratosis pilaris. It is often described in children with underlying xerosis, atopy, or ichthyosis vulgaris. Treatment is marginally effective and only provides temporary relief. [2]

Images of keratosis pilaris are below (also see Physical Examination).

Keratosis pilaris is theorized to result from defective keratinization of the follicular epithelium, resulting in the lack of proper desquamation of keratinocytes and the formation of a keratotic plug at the follicular orifice. Alternatively, some experts suggest that keratosis pilaris may not be a primary disorder of keratinization, but rather a disorder of the hair shaft. Circular hair shafts rupture the follicular epithelium, leading to inflammation and abnormal follicular keratinization. [3] More recently, atrophy or absence of sebaceous glands has been identified as an early feature in keratosis pilaris pathogenesis. [4]

Autosomal dominant inheritance has been described.

The etiology of keratosis pilaris is not completely known. Keratosis pilaris may be due to a disorder of corneocyte adhesion that prevents normal desquamation in the area around the hair follicle. It is often associated with xerosis, ichthyosis vulgaris, and atopy. Other conditions reportedly associated with keratosis pilaris include ichthyosis follicularis, ectodermal dysplasia, keratitis ichthyosis deafness (KID) syndrome, Down syndrome, and cardiofaciocutaneous syndrome.

Al-Maawali et al propose that the gene BTG1 is critical for the development of the distinctive keratosis pilaris observed in patients with interstitial deletion of 12q21-q22. [6]

Keratosis pilaris–like eruptions have been described in patients receiving targeted cancer therapies such as RAF inhibitors (ie, vemurafenib) and the Bcr-Abl tyrosine kinase inhibitor nilotinib. [7, 8, 9]

Keratosis pilaris is a very common disorder observed worldwide that affects approximately 42% of individuals. Some studies estimate that keratosis pilaris affects 50-80% of all adolescents.

The frequency of keratosis pilaris is increased in individuals with ichthyosis vulgaris, estimated at 74%. [5] Many older reports claim an increased incidence in patients with atopic dermatitis, but more recent studies have not demonstrated this association. Given a high prevalence and intensity of keratosis pilaris noted during puberty and in women with hyperandrogenism, some experts postulate that keratosis pilaris may be influenced by hormonal changes.

Keratosis pilaris has no known racial predilection.

Keratosis pilaris affects both males and females. The inflammatory form of keratosis pilaris may be more prevalent in females.

Keratosis pilaris most commonly develops during the first decade of life. It is estimated that 51% of cases are diagnosed during this period, while 35% are diagnosed during the second decade, 12% in the third decade, and 2% in the fourth decade.

Keratosis pilaris is a benign disorder and is not associated with increased mortality or long-term health consequences. Many patients find the lesions cosmetically unappealing and therefore seek treatment. A significant inflammatory component may be present, resulting in pruritus. Occasionally, keratosis pilaris may become secondarily infected due to scratching or abrasive self-therapy, in which case treatment of the infection is necessary.

The overall prognosis for patients is very good. Keratosis pilaris tends to improve over time, though it can persist with a waxing and waning course in some. Keratosis pilaris does not tend to leave scars.

A study performed by Poskitt demonstrated the following course [2] :

The condition dramatically improves in approximately 35% of patients, usually by late adolescence (mean age of improvement is 16 yrs).

The condition remains unchanged from the time of diagnosis in approximately 43% of patients.

Approximately 20% of patients experience a worsening of symptoms over time.

Approximately 50% experience a worsening of symptoms during wintertime, but only 60% of those who worsen improve over summertime.

Reassurance and gentle skin care are the most important recommendations the clinician can offer.

Hwang S, Schwartz RA. Keratosis pilaris: a common follicular hyperkeratosis. Cutis. 2008 Sep. 82(3):177-80. [Medline].

Poskitt L, Wilkinson JD. Natural history of keratosis pilaris. Br J Dermatol. 1994 Jun. 130(6):711-3. [Medline].

Thomas M, Khopkar US. Keratosis pilaris revisited: is it more than just a follicular keratosis?. Int J Trichology. 2012 Oct. 4(4):255-8. [Medline].

Gruber R, Sugarman JL, Crumrine D, Hupe M, Mauro TM, Mauldin EA, et al. Sebaceous gland, hair shaft, and epidermal barrier abnormalities in keratosis pilaris with and without filaggrin deficiency. Am J Pathol. 2015 Apr. 185 (4):1012-21. [Medline].

Mevorah B, Marazzi A, Frenk E. The prevalence of accentuated palmoplantar markings and keratosis pilaris in atopic dermatitis, autosomal dominant ichthyosis and control dermatological patients. Br J Dermatol. 1985 Jun. 112(6):679-85. [Medline].

Al-Maawali A, Marshall CR, Scherer SW, Dupuis L, Mendoza-Londono R, Stavropoulos DJ. Clinical characteristics in patients with interstitial deletions of chromosome region 12q21-q22 and identification of a critical region associated with keratosis pilaris. Am J Med Genet A. 2014 Mar. 164(3):796-800. [Medline].

Macdonald JB, Macdonald B, Golitz LE, LoRusso P, Sekulic A. Cutaneous adverse effects of targeted therapies: Part II: Inhibitors of intracellular molecular signaling pathways. J Am Acad Dermatol. 2015 Feb. 72 (2):221-36; quiz 237-8. [Medline].

Leong WM, Aw CW. Nilotinib-Induced Keratosis Pilaris. Case Rep Dermatol. 2016 Jan-Apr. 8 (1):91-6. [Medline].

Tawil MH, El Khoury R, Tomb R, Ghosn M. Nilotinib-induced Keratosis Pilaris Associated with Alopecia Areata and Eyebrow Thinning. Int J Trichology. 2017 Apr-Jun. 9 (2):87-89. [Medline].

Marqueling AL, Gilliam AE, Prendiville J, Zvulunov A, Antaya RJ, Sugarman J. Keratosis pilaris rubra: a common but underrecognized condition. Arch Dermatol. 2006 Dec. 142(12):1611-6. [Medline].

Castela E, Chiaverini C, Boralevi F, Hugues R, Lacour JP. Papular, profuse, and precocious keratosis pilaris. Pediatr Dermatol. 2012 May-Jun. 29(3):285-8. [Medline].

Sallakachart P, Nakjang Y. Keratosis pilaris: a clinico-histopathologic study. J Med Assoc Thai. 1987 Jul. 70(7):386-9. [Medline].

Novick NL. Practical management of widespread, atypical keratosis pilaris. J Am Acad Dermatol. 1984 Aug. 11(2 Pt 1):305-6. [Medline].

Gerbig AW. Treating keratosis pilaris. J Am Acad Dermatol. 2002 Sep. 47(3):457. [Medline].

Breithaupt AD, Alio A, Friedlander SF. A comparative trial comparing the efficacy of tacrolimus 0.1% ointment with Aquaphor ointment for the treatment of keratosis pilaris. Pediatr Dermatol. 2011 Jul-Aug. 28(4):459-60. [Medline].

Saelim P, Pongprutthipan M, Pootongkam S, Jariyasethavong V, Asawanonda P. Long-pulsed 1064-nm Nd:YAG laser significantly improves keratosis pilaris: a randomized, evaluator-blind study. J Dermatolog Treat. 2012 Mar 4. [Medline].

Schoch JJ, Tollefson MM, Witman P, Davis DM. Successful Treatment of Keratosis Pilaris Rubra with Pulsed Dye Laser. Pediatr Dermatol. 2016 Jul. 33 (4):443-6. [Medline].

Derek H Chu, MD Clinical Assistant Professor, Department of Dermatology, Stanford University School of Medicine

Derek H Chu, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Medical Association, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Andrea Leigh Zaenglein, MD Professor of Dermatology and Pediatrics, Department of Dermatology, Hershey Medical Center, Pennsylvania State University College of Medicine

Andrea Leigh Zaenglein, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Received consulting fee from Galderma for consulting; Received consulting fee from Valeant for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Anacor for consulting; Received grant/research funds from Stiefel for investigator; Received grant/research funds from Astellas for investigator; Received grant/research funds from Ranbaxy for other; Received consulting fee from Ranbaxy for consulting.

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.

Steven J Escobar, MD Staff, Division of Pulmonary and Critical Care Medicine, Naval Medical Center, San Diego

Steven J Escobar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Pediatric Keratosis Pilaris

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