Pityriasis Rubra Pilaris Medication
- Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD more...
Medication Summary
The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Due to the rarity of this disease, therapy has been based on anecdotal reports.[20] No large controlled trials have been performed. Infliximab has been reported anecdotally to be of benefit, as has etanercept.[21, 22, 23, 24]
Retinoids
Class Summary
A study of 12 patients by Dickens revealed that 80% of the patients had improvement from the use of oral retinoids. Clinical improvement can be expected within 4-6 months.[25]
Acitretin (Soriatane)
Metabolite of etretinate and related to both retinoic acid and retinol (vitamin A). Mechanism of action unknown. However, thought to exert therapeutic effect by modulating keratinocyte differentiation, keratinocyte hyperproliferation, and tissue infiltration by inflammatory cells. Approved for treatment of severe psoriasis.
Isotretinoin (Accutane)
Synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Approved for use in severe recalcitrant nodular acne. Recent review by Allison et al revealed clearing in 5 of 6 pediatric patients with pityriasis rubra pilaris within 6 mo.
A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.
Antimetabolites
Class Summary
These agents inhibit cell growth and proliferation. May also cause immunosuppression.[26]
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in immunosuppression. Approved for use in transplantation patients and patients with rheumatoid arthritis.
Methotrexate (Rheumatrex)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction. Successful treatment reported. Follow same guidelines as for use in psoriasis. Improvement may occur in 6 wk; complete response after 3-4 mo. Relapse may occur upon discontinuation.
Immunosuppressants
Class Summary
These agents inhibit key factors that regulate the immune system. Case reports have shown benefit in some patients with pityriasis rubra pilaris.[27]
Cyclosporine (Neoral, Sandimmune)
Cyclic polypeptide immunosuppressant agent produced as a metabolite by the fungus species Beauvaria nivea. Approved for use in organ transplantation patients, rheumatoid arthritis, and psoriasis.
Biologic agents
Class Summary
The use of monoclonal antibodies that suppress the immune system may improve the clinical aspects of the disease.[28] A case series has compared infliximab with etanercept and found a more rapid onset of action with infliximab but roughly equal treatment duration required when compared with etanercept.[29] Adalimumab has been added to the list,[30] as has ustekinumab.[31]
Etanercept (Enbrel)
Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses.
Infliximab (Remicade)
Chimeric monoclonal antibody that binds specifically to human tumor necrosis factor-alpha. Approved for treatment of rheumatoid arthritis, Crohn disease, ankylosing spondylitis, and psoriatic arthritis. Several reported cases of adult-onset pityriasis rubra pilaris with excellent responses to infliximab.
Adalimumab (Humira)
Recombinant human IgG1 monoclonal antibody specific for human TNF. Binds specifically to TNF-alpha and blocks interaction with p55 and p75 cell-surface TNF receptors. This interferes with cytokine driven inflammatory processes. It also lyses surface TNF-expressing cells in vitro in the presence of complement, but it does not bind to TNF-beta (lymphotoxin).
Ustekinumab (Stelara)
Ustekinumab is a human monoclonal antibody directed against IL-12 and IL-23, thereby interfering with T-cell differentiation and activation and subsequent cytokine cascades.
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