Published on 18/03/2015 by admin
Filed under Dermatology
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Nisha C. Desai and Robert A. Silverman
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Keratosis pilaris (KP) is a common inherited disorder of unknown etiology characterized by tenacious keratin plugging of follicular orifices in specific locations on the body. It typically becomes apparent during childhood on the extensor aspect of the upper arms, anterior surface of the thighs, and lateral aspects of the cheeks. In severe cases, it may extend onto the distal extremities, shoulders, and buttocks. Perifollicular erythema and a background of erythema on the cheeks is not uncommon (keratosis pilaris rubra, KPR). Variants of KP include keratosis pilaris atrophicans (ulerythema ophryogenes), keratosis follicularis spinulosa decalvans (KFSD), folliculitis spinulosa decalvans (FSD), and atrophoderma vermiculatum; these are also addressed in this chapter. KP is a common, though minor < feature of patients with atopic dermatitis. It may be exacerbated during pregnancy and is a feature of infants with malnutrition. Extensive or persistent disease may be observed in patients with Down syndrome, and cardiofaciocutaneous syndrome among others. KP has also been observed in patients treated with vemurafenib.
KP is usually asymptomatic and often does not require treatment. Those with extensive or symptomatic involvement often desire treatment, though, even without therapy, the condition often becomes less prominent with increasing age.
Initial treatment involves measures to decrease excessive skin roughness and follicular accentuation that often waxes and wanes over a period of months. Harsh soaps are to be avoided, but cleansers containing 2% salicylic acid or glycolic acid may be helpful. Bathing should be followed by application of an emollient to damp skin. Keratolytic agents such as glycolic acid, ammonium lactate, salicylic acid, and urea containing humectants are the mainstays of treatment. Salicylic acid 2% in 20% urea cream or salicylic acid 6% in propylene glycol combines the properties of an emollient with a keratolytic agent. Twice daily application of one of these compounds for at least a 3-week trial is recommended. The addition of gentle massage with a polyester sponge such as a Buf-Puf® during a shower or bath is particularly helpful. Vigorous scowering can lead to irritation. Once adequate relief of symptoms has been achieved, maintenance therapy of weekly or twice-weekly application of 20% urea cream (Carmol 20®) is recommended.
Topical retinoids may be tried in some cases but can be irritating and expensive if large areas are treated. Lower concentrations of topical tretinoin such as 0.025% cream can be used at first. If there is no significant irritation, higher-concentration preparations can be applied. Tazarotene 0.05% cream has also been shown to decrease the pruritus, erythema, and roughness of KP in a recent placebo-controlled trial.
If a significant inflammatory component is present, the inflammation can be treated for defined, short periods with a medium potency topical corticosteroid in an emollient base. Once inflammation has abated, corticosteroids are discontinued and keratolytics are introduced. With the presence of pustules, suggestive of a bacterial infection, topical antibiotics, such as clindamycin lotion, can be effective. A 3-month course of oral vitamin A, 50 000 units three times a day, has been advocated for some patients. Oral isotretinoin has been helpful in some patients with ulerythema ophryogenes and atrophoderma vermiculatum. In the author’s experience, calcipotriol and calcineurin inhibitors have not been helpful. In one small double blind study, Aquaphor™ improved KP as much as topical tacrolimus.
Cutaneous laser therapy has been utilized for several variants of KP. Pulsed-dye laser (PDL) treatment has recently been demonstrated to be a safe treatment for the erythema associated with keratosis pilaris atrophicans and KPR. Long pulsed 1064 nm Nd:YAG laser also significantly improved KP in a single blinded observational study. Potassium titanyl phosphate (KTP) laser has also cleared the keratotic papules and reduced the erythema of KPR lesions. However, reports involved only a few cases and made no mention of sustained long-term effectiveness. KPSD has been treated with laser-assisted hair removal using a normal mode, non-Q-switched, high-energy, pulsed ruby laser. This mode of treatment has been effective in producing persistent reductions of inflammation in this KP variant, albeit at the expense of permanent hair loss in treated areas.
Serum testosterone levels (in obese females)
Complete physical examination
Ophthalmologic examination
Pavlović MD, Milenković T, Dinić M, Misović M, Daković D, Todorović S, et al. Diabetes Care 2007; 30: 1964–7.
The presence and frequency of skin manifestations were examined and compared in 212 unselected type 1 diabetic patients and 196 healthy sex- and age-matched control subjects. KP affected 12% of the diabetic patients compared with 1.5% of control subjects. Significant association was also found between acquired ichthyosis and KP.
Other conditions associated with KP include high body mass index, dry scaly legs, atopic conditions, pregnancy, cardiofaciocutaneous syndrome, and hyperandrogenism in obese females. KP-like lesions have been observed in patients treated with vemurafenib for metastatic melanoma.
Thomsen K, Nyfors A. Arch Dermatol 1973; 107: 629–30.
Case report of a patient who suddenly developed KP and was diagnosed with Hodgkin’s disease 6 months later. Successful chemotherapy of the Hodgkin’s disease was followed by clearance of the skin lesions.
This case may be analogous to cases of acquired ichthyosis reported in association with Hodgkin’s disease. On the other hand, KP is very prevalent and can remit spontaneously, so linkage with other conditions and purported responses to therapy of those conditions should be interpreted with care.
Oranje AP, van Osch LD, Oosterwijk JC. Arch Dermatol 1994; 130: 500–2.
Patients with keratosis pilaris atrophicans are reported to have increased incidence of ocular abnormalities, including photophobia, corneal deposits, juvenile cataracts, and corneal dystrophy.
This cogent review clarifies the confusing nosology of KP variants.
Weber TM, Kowcz A, Rizer R. J Am Acad Dermatol 2004; 50(Suppl 1): 47.
A formulation containing sodium lactate and urea was tested on 32 subjects with mild to severe keratosis pilaris. The authors reported progressive, statistically significant improvements in overall condition, skin roughness, and skin tone, at 3, 6, and 12 weeks of use.
Diamant F. Clin Ther 1980; 3: 250–3.
Seven patients who used polyester sponges from three times per week to once daily for their keratosis pilaris improved after a mean treatment duration of 7.4 weeks.
Novick NL. J Am Acad Dermatol 1984; 11: 305–6.
Thirty patients with widespread, atypical or psychologically troubling KP were treated according to a protocol that included prevention of excessive skin drying, application of salicylic acid 2–3% in 20% urea cream using a polyester sponge, and use of emollient-based topical corticosteroids if a prominent inflammatory component was present. All patients reported satisfaction with cosmetic results. Clearing of lesions was noted in 75–100% of cases, with elimination of most lesions achieved within 2 to 3 weeks of daily therapy.
Drago F, Maietta G, Parodi A, Rebora A. Clin Exp Dermatol 1993; 18: 45–6.
Case report of a patient with keratosis pilaris decalvans non-atrophicans with follicular keratotic papules on the limbs and trunk accompanied by loss of body hair. The patient was successfully treated over the course of 3 months with topical emollients and multivitamins. The KP completely disappeared, leaving no scars, and there was complete regrowth of hair.
Traditional methods of treating uncomplicated KP seem to be effective in treating cases of keratosis pilaris decalvans non-atrophicans.
Breithaupt AD, Alio A, Friedlander SF. Pediatr Dermatol 2011; 28: 459–60.
This was a bilateral paired comparison double blind study which showed improvement but no difference between Aquaphor™ and tacrolimus 0.1%.
Bogle MA, Ali A, Bartel H. J Am Acad Dermatol 2004; 50(Suppl 1): 39.
A randomized, placebo-controlled, double-blind prospective study of tazarotene 0.05% cream for the treatment of KP on the posterior arms of 33 patients reportedly resulted in statistically significant improvement in pruritus, erythema, and roughness of KP lesions.
Poskitt L, Wilkinson JD. Br J Dermatol 1994; 130: 711–13.
This retrospective questionnaire study of 49 patients with KP yielded 14 patients who had benefited from a variety of treatments. Of these patients, eight reported therapy with topical tretinoin cream to be helpful.
Baden HP, Byers HR. Arch Dermatol 1994; 130: 469–75.
Twenty-one patients with keratosis pilaris atrophicans were treated with various agents and combinations of agents, including keratolytics, antibiotics, topical corticosteroids, and retinoids, all with very limited response. Treatment of four patients with isotretinoin 1mg/kg resulted in little or no improvement in three patients and exacerbation of the condition in one patient.
Weightman W. Clin Exp Dermatol 1998; 23: 89–91.
Atrophoderma vermiculatum is a rare variant of KP that results in reticular or honeycomb scarring of the face. In this case report, isotretinoin induced a remission in the inflammatory component of the disease, which was maintained after cessation of treatment.
In severe cases of atrophoderma vermiculatum with significant scarring, a trial of isotretinoin therapy is worthwhile to halt progression of the disease.
Frosch PJ, Brumage MR, Schuster-Pavlovic C, Bersch A. J Am Acad Dermatol 1988; 18: 538–42.
In this variant of KP, a symmetric worm-eaten or reticular atrophy may result. Dermabrasion may be used to reduce the eventual scarring.
Layton AM, Cunliffe WJ. Br J Dermatol 1993; 129: 645–6.
Ulerythema ophryogenes, a variant of KP, begins in the eyebrows as small, discrete, horny, pinhead-sized papules at the hair follicle orifices that spread to the forehead and scalp. Atrophy and alopecia of the eyebrows and scalp may result. There is a variable response to isotretinoin.
Marqueling AL, Gilliam AE, Prendiville J, Zvulunov A, Antaya RJ, Sugarman J, et al. Arch Dermatol 2006; 142: 1611–6.
In this case series from dermatology practices in the US, Canada, Israel, and Australia, the clinical characteristics of 27 patients with KPR are described. Treatments given for KPR in these patients included emollients; urea, lactic acid, topical corticosteroids, or a combination of these ingredients; topical agents containing cholecalciferol, topical or systemic retinoid agents; topical corticosteroids; and topical salicylic acid. In most patients, there was no substantial improvement with these treatments. One patient had excellent response with PDL.
Two patients responding to a retrospective questionnaire stated that treatment with tetracyclines had ‘dramatically’ improved their KP. The authors warned that the questionnaire did not permit exclusion of possible coincidental acne in these patients and that their reported improvement may have been secondary to the known therapeutic effects of tetracycline on acne. However, tetracyclines are recognized anti-inflammatory agents and this may explain the improvements in KP that are sometimes noted with these agents.
Chui CT, Berger TG, Price VH, Zachary CB. Dermatol Surg 1999; 25: 34–7.
A patient with KPSD of the scalp complicated by recurrent secondary infection was treated with a variety of oral antibiotics and topical corticosteroids with limited success. The patient subsequently underwent five treatments with a normal mode, non-Q-switched, high-energy, pulsed ruby laser at fluences of 19–21 J/cm2 at 6-week intervals. Eight months after initiation of treatment, significant reduction of inflammation occurred in treated areas, at the expense of a persistent diminution of hair growth.
This is a promising mode of therapy for patients with severe or symptomatic conditions who have not responded to other modes of therapy and are willing to accept permanent hair loss on treated areas as a side effect.
Kunte C, Loeser C, Wolff H. J Am Acad Dermatol 1998; 39(5 Pt 2): 891–3.
Case report of a patient with folliculitis spinulosa decalvans with keratotic follicular papules on the trunk, extremities, and lateral, alopecic part of the eyebrows and erythema, scaling, and follicular hyperkeratoses on the scalp. Isotretinoin and topical corticosteroids were ineffective. Dapsone (100 mg/day) led to the resolution of inflammation and pustulation on the scalp within 1 month. The scarring alopecia did not expand since the dapsone therapy.
Clark SM, Mills CM, Lanigan SW. J Cutan Laser Ther 2000; 2: 151–6.
All facial areas involved with keratotis pilaris atrophicans in 12 patients were treated with the PDL at 585 nm. Patients received two to eight treatments with the laser, with energies ranging from 6.0 to 7.5 J/cm2. The authors report clinical improvement in all patients, with significant reduction in erythema scores. Significant improvement was not achieved in skin roughness. Treatment was generally well tolerated, with side effects mostly limited to local pain during treatment.
Dawn G, Urcelay M, Patel M, Strong AMM. Br J Dermatol 2002; 147: 822–4.
A 15-year-old girl with erythematous papules on her cheeks, eyebrows, and chin was treated with a KTP laser (532 nm) with energy fluence of 12–14 J/cm2 and pulse width of 510 ms. Both cheeks were treated seven times at 6- to 8-week intervals, resulting in good cosmetic clearance of keratotic papules and gradual reduction of erythema on her face. During a follow-up period, the patient required two treatments at 4-month intervals, and was happy with her cosmetic result.
Kaune KM, Haas E, Emmet S, Schvan MP, Zutt M. Dermatol Surg 2009; 35: 1592–5.
Alcántara González J, Boixeda P, Truchuelo Díez MT, Fleta Asín B. J Eur Acad Dermaol Venereol 2011; 25: 710–14.
Saelim P, Pongprutthipan M, Pootongkam S, Jariyasethavong V, Asawanonda P. J Dermatolog Treat 2012; [Epub ahead of print].
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Sodium lactate and urea cream
Polyester sponge
Salicylic acid in urea
Topical corticosteroids
Topical tazarotene
Topical tretinoin
Isotretinoin
Topical tacrolimus
Aquaphore
Dermabrasion (for atrophoderma vermiculatum)
Tetracyclines: oxytetracycline, minocycline
Long-pulse, non-Q-switched, ruby laser (for keratosis pilaris spinulosa decalvans)
Dapsone (for keratosis folliculitis decalvans)
Pulsed tunable dye laser (for keratosis pilaris atrophicans)
Potassium titanyl phosphate laser (for keratosis rubra pilaris)
