Published on 16/03/2015 by admin
Filed under Dermatology
Last modified 22/04/2025
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Anne-Marie Tobin and Brian Kirby
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Pityriaisis rubra pilaris is a rare papulosquamous disorder characterized by erythroderma, orange-red keratoderma, and ‘islands of sparing’. It can divided into six subtypes based on morphological phenotype, age of onset, and presence of HIV infection:
Type I: classical adult; rapid onset erythroderma, scale and keratoderma in a craniocaudal pattern
Type II: atypical adult pattern
Type III: classical juvenile pattern
Type IV: atypical juvenile pattern
Type V: well-circumscribed pattern
Type VI: HIV-associated pityriasis rubra pilaris.
More than 50% of cases are of the classical type I adult onset pityriasis rubra pilaris. The majority of cases of type I classical pityriasis rubra pilaris go into spontaneous remission after 1 to 3 years.
As pityriasis rubra pilaris is a rare condition, there are no randomized controlled trials assessing treatment efficacy. Treatment recommendations are based on case reports and small case series. Pityriasis rubra pilaris has been reported in association with malignancy. These reports have been inconsistent and suggest a chance occurrence rather than a true association.
Topical therapies are primarily used as anti-pruritic agents. Emollient use should be encouraged in combination with emollient baths. Topical steroids may reduce pruritus but are often ineffective.
There are a few case reports of effective use of both narrowband UVB and PUVA for pityriasis rubra pilaris. Pityriasis rubra pilaris can often be exacerbated by UV light and we recommend that narrowband UVB and PUVA are only used cautiously and only when other treatments are contraindicated.
Oral retinoids have been the mainstay of therapy for classical type I and type III pityriasis rubra pilaris. Doses of 0.5 mg/kg of acitretin and upwards are required for adequate control. This often results in significant mucocutaneous side effects and hair loss. Skin fragility and pruritus are common side effects at these doses. It is our practice to initiate treatment with acitretin for rapid improvement but to add in a second oral agent (usually methotrexate) if intolerable side effects ensue.
Methotrexate has been reported in several case series as being effective in classical type I pityriasis rubra pilaris. The long-term efficacy of methotrexate is well established. Doses of up to 30 mg weekly are necessary and concomitant folic acid is recommended. Despite reservations of its use with acitretin regarding hepatotoxicity it has been reported as a safe combination and we would concur with that view.
Due to its similarity to psoriasis, pityriasis rubra pilaris has been treated with TNF-α inhibitors. The use of TNF inhibitors appears effective in the treatment of pityriasis rubra pilaris and has been the subject of an excellent systematic review.
Histology
HIV testing
Histology of the affected skin may be useful in aiding the diagnosis. It is important to obtain histology to rule out other causes of erythroderma, especially cutaneous T-cell lymphoma.
Routine bloods and chest X-ray are recommended as markers of general health and as screening for the use of systemic agents. An HIV test should be done in all patients.
Dicken CH. J Am Acad Dermatol 1994; 31: 997–9.
Fifteen patients were treated with isotretinoin; 10 had a complete response and two had partial responses. Six patients had etretinate; four had a complete response.
There are few reports of the use of acitretin in pityriasis rubra pilaris although in practice it has superseded both isotretinoin and etretinate.
Klein A, Landthaler M, Karrer S. Am J Clin Dermatol 2010; 11: 157–70.
Griffiths WAD. Clin Exp Dermatol 1980; 5: 105–12.
Methotrexate treatment of pityriasis rubra pilaris is discussed in these papers. In one series, methotrexate was effective in 17 of 42 patients only. This was a retrospective report where various doses and dosing regimens were used. It is the authors’ view that doses up to 30 mg weekly may be needed for control.
Petrof G, Almaani N, Archer CB, et al. J Eur Acad Dermatol Venereol 2012 [Epub ahead of print].
Barth D, Harth W, Treudler R, Simon JC. J Dtsch Dermatol 2009; 7: 1071–4.
Eighty-three percent of 15 patients achieved a complete response. In most of these cases the remission achieved was attributable to TNF inhibition. Patients who received infliximab received 5 mg/kg at zero, two, six and then every 8 weeks (the same as is commonly used for psoriasis). The majority of patients who have been treated with TNF inhibitors have type I classic adult pityriasis rubra pilaris. The successful use of infliximab and methotrexate in combination has also been reported.
Gonzalez-Lopez A, Velasco E, Pozo T, Del Vilar A. Br J Dermatol 1999; 140: 931–4.
Pityriasis rubra pilaris associated with HIV infection may respond to antiretroviral therapy. The response appears to correlate with a reduction in viral load.
Kirby B, Watson R. Br J Dermatol 2000; 142: 376–7.
This is a single case report in a case of juvenile classic pityriasis rubra pilaris (type III).
Herbst RA, Vogelbruch M, Ehnis A, Kapp A, Weiss J. Br J Dermatol 2000; 142: 574–5.
This is a case report of long-wave UVA and retinoid in combination for the treatment of classic type I pityriasis rubra pilaris.
Evangelou G, Murdoch SR, Palamaras I, Rhodes LE. Photodermatol Photoimmunol Photomed 2005; 21: 272–4.
Pityriasis rubra pilaris may be aggravated by UV light and these treatments should be considered only when other more effective therapies are ineffective or contraindicated.
Hofer A, Mullegger R, Kerl H. Arch Dermatol 1999; 135: 475–6.
Haenssle HA, Bertsch HP, Wolf C, Zutt M. Clin Exp Dermatol 2004; 29: 244–6.
These are single case reports of the efficacy of extracorporeal photochemotherapy in pityriasis rubra pilaris.
Kerr AC, Ferguson J. Br J Dermatol 2007; 156: 1055–6.
This is a report of the successful use of IVIG at a dose of 2 g/kg given over 3 days. The dose was repeated every 4 weeks. The patient had failed therapy with anti-TNF agents.
Ruiz-Villavarde R, Sanchez-Cano D Eur J Dermatol 2010; 20: 630–1.
Wohirab J, Kreft B.. Br J Dermatol 2010; 163: 655–6.
There are two case reports of the successful use of ustekinumab in type I pityriasis rubra pilaris. The doses used were similar to those used in psoriasis.
Coras B, Vogt TH, Ulrich H, Landthaler M, Hohenleutner U. Br J Dermatol 2005; 152: 388–99.
Despite their widespread use in psoriasis, we can find only one case report of fumaric acid ester use in pityriasis rubra pilaris. This was the successful use of fumaric acid esters in a patient with atypical juvenile (type IV) pityriasis rubra pilaris.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Retinoids
Methotrexate
TNF-α inhibitors
Retroviral therapy
Acitretin and narrowband UVB
Acitretin and UVA1
Intravenous immunoglobulin
Extracorporeal photochemotherapy
Fumaric acid esters
Ustekinumab
