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

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

Coauthor(s): Steven J Escobar, MD, Fellow, Division of Pulmonary and Critical Care Medicine, Naval Medical Center, San Diego

Editors: Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

Synonyms and related keywords: keratosis pilaris alba, keratosis pilaris rubra, goose bumps, hair follicle, keratinized hair follicles, keratotic papule, perifollicular erythema, ichthyosis vulgaris, atopic dermatitis, hyperandrogenism, erythematous papules, corneocyte adhesion, hyperkeratosis, hypogranulosis

Background

Keratosis pilaris is an extremely common and benign disorder of keratinized hair follicles. The disease is characterized by grouped, horny, keratotic follicular papules predominantly located on the extensor surfaces of the proximal limbs, most commonly of the posterolateral upper arms and anterior thighs. It is usually asymptomatic except for its cosmetic appearance. Treatment is marginally effective and only provides temporary relief.

Pathophysiology

Apparently because of lack of proper desquamation of keratinocytes, the follicular orifice becomes plugged with keratin and results in a keratotic papule. A variable degree of perifollicular erythema occurs.

Frequency

United States

Significant individual variation is observed in the prominence and severity of keratosis pilaris, which affects 42% of the population. Some studies estimate that keratosis pilaris affects 50-80% of all adolescents. The disorder has a familial relationship, which is consistent with autosomal dominant transmission.

Frequency is increased, reported at 74%, in individuals with ichthyosis vulgaris. Many older reports claim an increased incidence with atopic dermatitis, but more recent studies do not demonstrate this association. Hormonal influence may occur because a high prevalence and intensity of keratosis pilaris is noted during puberty and in women with hyperandrogenism.

International

Incidence is similar to that observed in the United States.

Mortality/Morbidity

Keratosis pilaris is a benign disorder; treatment in most cases requires simple reassurance and general skin care recommendations. Many patients find lesions cosmetically unappealing and, therefore, seek treatment. Occasionally, they may become secondarily infected because of scratchy, tight-fitting clothing or abrasive self-therapy, in which case treatment of the infection is necessary. A significant inflammatory component may be present and may be relieved with topical steroid therapy. Treatment of the noninflamed horny papules can be difficult because they have proven resistant to most modes of therapy.

Race

No evidence of racial predilection exists.

Sex

The inflammatory form of keratosis pilaris is more prevalent in females.

Age

Fifty-one percent of cases are diagnosed in people in the first decade of life, 35% in the second, 12% in the third, and 2% in the fourth.



History

  • The patient may report groups of keratotic papules, which feel rough and prickly. The patient may describe them as persistent, rough goose bumps. They are not painful or significantly pruritic in most patients.
  • About half of all affected patients notice a worsening of symptoms in the winter months.
  • These lesions tend to improve after a few years.

Physical

  • Keratosis pilaris alba is the more common variant and is characterized by small gray-white papules with a negligible inflammatory component.
  • Keratosis pilaris rubra has a significant inflammatory component, and thus patients present with small erythematous papules. More widespread areas of skin are involved. This variation is most conspicuous during the winter months.
  • Observe for small, horny, follicular papules with (ie, rubra) or without (ie, alba) surrounding erythema.
  • Most commonly, lesions occur on the posterolateral upper arms and anterior thighs. Less commonly, lesions involve the face, buttocks, and trunk.
  • In involved areas, lesions are extensive, monomorphic, and very evenly spaced.
  • A fine hair may pierce the papules, or hair may be found coiled up within the keratin plug.
  • The keratin plug cannot be expressed with pressure.

Causes

Etiology is unknown, although it may be due to a disorder of corneocyte adhesion that prevents normal desquamation in the area around the follicle.



Other Problems to be Considered

Acne vulgaris
Pityriasis rubra pilaris
Folliculitis - Bacterial, fungal, or irritant
Lichen spinulosus
Phrynoderma
Keratosis pilaris atrophicans
Lichen nitidus
Disseminate and recurrent infundibulofolliculitis
Traumatic anserine folliculosis
Cutis anserina (gooseflesh)
Follicular atopic dermatitis

The papules of lichen nitidus tend to be flatter, and lesions develop in crops. Lichen nitidus also shows the property of koebnerization. Keratosis pilaris does not koebnerize.

Disseminate and recurrent infundibulofolliculitis is an uncommon slightly pruritic papular follicular eruption that affects the neck, face, trunk, and proximal extremities and occurs almost exclusively in black people. Disseminate and recurrent infundibulofolliculitis differs from keratosis pilaris in distribution and is less keratotic.



Lab Studies

  • Keratosis pilaris is clinically diagnosed by the appearance and distribution of the lesions.

Histologic Findings

The epidermis demonstrates a fine lamellar follicular plug arranged concentrically around a normal hair shaft. The plug extends to the level of the hair follicle, and one or more rudimentary hairs may also be present. Mild hyperkeratosis and hypogranulosis may also be noted.



Medical Care

  • Education and reassurance are the cornerstones of therapy for keratosis pilaris.
  • The noninflamed horny papules usually remit with age and increasing time, but they are resistant to most forms of short-term therapy.
  • Encourage tepid showers instead of hot baths, along with the use of mild soaps and a home humidifier.
  • An emollient cream may help alleviate rough surfaces in mild cases. A topical keratolytic agent such as lactic acid, salicylic acid, or urea preparations may be beneficial in more extensive cases. Several recent reports claim good results with 2-3% salicylic acid in 20% urea cream. Topical tretinoin therapy has also been used with varying degrees of success.
  • Lesions with significant inflammation may improve with the use of medium-potency emollient-based topical steroid preparations. Inflammation is usually reduced markedly by 7 days, at which point the steroid should be discontinued.



Drug Category: Alpha hydroxy acids

Alpha hydroxy acid is a normal constituent of tissues and blood. Acids act as humectants when applied topically and may decrease corneocyte cohesion.

Drug NameAmmonium lactate cream (Lac-Hydrin cream 12%)
DescriptionEmollient available in 225-g and 400-g bottles and promotes hydration and removal of excess keratin. Contains lactic acid, an alpha hydroxy acid that has keratolytic action, thus facilitating release of comedones.
Adult DoseApply to affected area bid/tid
Application to the skin while moist after washing or bathing enhances the moisturizing effect
Pediatric DoseApply as in adults
ContraindicationsDocumented hypersensitivity
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsApply hydration, moisturizers, and possibly topical corticosteroids until fissures and inflammation are reduced; transient stinging, burning, erythema, and peeling have been noted after application of ammonium lactate lotion, especially when applied to abraded, inflamed, or irritated skin

Drug Category: Emollients containing urea

Topically applied urea has a hygroscopic effect by increasing the water retention in skin and it decreases pruritus.

Drug NameUrea cream 20% (Carmol 20, Ureacin, Lanaphilic)
DescriptionApplication of 20% urea promotes hydration and removal of excess keratin.
Adult DoseApply to affected area bid/tid
Application to the skin while moist after washing or bathing enhances the moisturizing effect
Pediatric DoseApply as in adults
ContraindicationsDocumented hypersensitivity
InteractionsNone reported with topical use
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsApply hydration, moisturizers, and possibly topical corticosteroids until fissures and inflammation are reduced; transient stinging, burning, erythema, and peeling have been noted after application of 20% urea, especially when applied to abraded, inflamed, or irritated skin

Drug Category: Gels containing salicylic acid

These agents produce desquamation of the skin's horny layer. They are keratolytic at concentrations of 2-6%.

Drug NameSalicylic acid 6% (Keralyt gel)
DescriptionRemoves excess keratin.
Adult DoseApply a thin layer to affected area qd/bid
Pediatric DoseApply as in adults
ContraindicationsDocumented hypersensitivity; prolonged use in infants, patients with diabetes mellitus, and patients with impaired circulation (not recommended); use on moles, birthmarks or warts with hair growing from them, genital or facial warts or warts on mucous membranes, irritated skin, or any area infected or reddened
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsBurning and irritation at site of exposure may occur; avoid contact with mucous membranes and eyes; prolonged use over large areas, especially in children, may result in salicylate toxicity

Drug Category: Retinoids

Retinoic acid decreases cohesiveness of follicular epithelial cells, stimulates mitotic activity, and increases turnover of follicular epithelial cells.

Drug NameTretinoin (Retin-A 0.025% - 0.1% cream)
DescriptionReduces cohesion among keratinized cells.
Adult DoseBegin with lowest tretinoin concentration and increase as tolerated; apply hs or qod; decrease application frequency if irritation develops
Pediatric DoseApply as in adults
ContraindicationsDocumented hypersensitivity
InteractionsToxicity increases with coadministration of benzoyl peroxide, salicylic acid, and resorcinol; avoid topical sulfur, resorcinol, salicylic acid, other keratolytics, abrasives, astringents, spices, and lime
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsPhotosensitivity may occur with excessive sunlight exposure, wear sunscreen over exposed areas; during the first few wk, the patient may experience redness, burning, or peeling; most patients adapt to treatment; switch those who become excessively irritated to qod or q3d therapy; do not apply to mucous membranes, mouth, and angles of nose

Drug Category: Topical corticosteroids

These agents elicit anti-inflammatory and immunosuppressive properties.

Drug NameTriamcinolone acetonide 0.1% cream (Aristocort, Kenalog)
DescriptionA moderate-potency steroid with anti-inflammatory properties. It treats inflammatory dermatosis that is responsive to steroids. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult DoseApply sparingly to affected areas qd/bid until inflammatory component begins to resolve (typically 7 d)
Pediatric DoseApply as in adults with caution
ContraindicationsDocumented hypersensitivity; fungal, viral, and bacterial skin infections
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsAs with all topical corticosteroids, skin atrophy with telangiectasia, stria, purpura, and acne may occur; limit the use of topical steroids to the initial inflammatory treatment phase; long-term use of topical corticosteroids has no role in keratosis pilaris; limit tube size to reflect area and duration of treatment; do not use in decreased skin circulation; prolonged use, applications over large areas, and use of potent steroids and occlusive dressings may result in systemic absorption; systemic absorption may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria



Complications

  • Secondary bacterial infections can occur in traumatized lesions.

Prognosis

A 1994 study performed by Poskitt demonstrates the following course:1

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

Patient Education

  • Reassurance and general skin care are the most important recommendations the physician can offer.



Special Concerns

  • Frequency of keratosis pilaris is increased in individuals who have autosomal dominant ichthyosis vulgaris. Recent reports indicate that keratosis pilaris is most likely a phenotype of ichthyosis vulgaris.2 An association between keratosis pilaris and Noonan and Down syndromes is also reported.



Media file 1:  Although other sites may be involved, keratosis pilaris is most common on the lateral upper arms and upper thighs.
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Media type:  Photo

Media file 2:  Upon closer examination of keratosis pilaris, the lesions are very evenly spaced. This even distribution is consistent with the follicular origin of this disorder.
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Media type:  Photo

Media file 3:  Close examination of keratosis pilaris shows keratotic papules associated with hair follicles. Keratinocytes normally shed from the surrounding skin become dust on the floor. Keratinocytes at the follicular orifice are retained, producing these papules.
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Media type:  Photo

Media file 4:  Bacteria associated with the follicular papules of keratosis pilaris may cause some lesions to become erythematous or pustular.
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Media type:  Photo

Media file 5:  This patient has lichen nitidus. Lichen nitidus is a differential diagnosis for keratosis pilaris. The papules of lichen nitidus tend to be flatter, and lesions develop in crops. Etiology of lichen nitidus is unknown.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo

Media file 6:  Lichen nitidus also shows the property of koebnerization. Keratosis pilaris does not koebnerize.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo

Media file 7:  Disseminate and recurrent infundibulofolliculitis is an uncommon slightly pruritic papular follicular eruption that affects the neck, face, trunk, and proximal extremities. It is a differential diagnosis for keratosis pilaris and occurs almost exclusively in blacks. The cause is unknown, and treatment has been totally unsuccessful. Disseminate and recurrent infundibulofolliculitis differs from keratosis pilaris in distribution and is less keratotic.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Keratosis Pilaris excerpt

Article Last Updated: Dec 4, 2007