eMedicine Specialties > Dermatology > Papulosquamous Diseases
Pityriasis Rubra Pilaris: Treatment & Medication
Updated: May 22, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Topical medications
- Topical corticosteroids may provide some patient comfort, but they are believed to have little long-term therapeutic effect on pityriasis rubra pilaris (PRP).
- Calcipotriol is a vitamin D analogue that has been used in the topical treatment of psoriasis. A report of successful treatment has been documented in 3 patients with pityriasis rubra pilaris; however, controlled studies are needed to further assess its usefulness.15
- The topical retinoid tazarotene has been used for topical treatment of psoriasis and acne. It has been reported to improve juvenile circumscribed pityriasis rubra pilaris.16
- Emollients reduce fissuring and dryness, providing some patient comfort. Petroleum jelly or one of the many proprietary emollients may be used.
- Extracorporeal photochemotherapy: This involves the ex vivo exposure of leukapheresed peripheral blood mononuclear cells to UVA in the presence of 8-MOP (DNA-intercalating agent) and subsequent reinfusion of the treated cells. Successful treatment of a patient with pityriasis rubra pilaris that was unresponsive to standard treatments has been reported.17 Further studies are needed.
Medication
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.18 No large controlled trials have been performed. Infliximab has been reported anecdotally to be of benefit, as has etanercept.19,20,21,22
A clinical trial that is currently recruiting is A Pilot Study of Alefacept for the Treatment of Pityriasis Rubra Pilaris.
Retinoids
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.23
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.
Adult
Recommended dose: 25-50 mg PO qd; take with food
Pediatric
Not established
Methotrexate (MTX) (increased toxicity); interferes with effects of microdosed progestin minipill; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d); vitamin A (increased toxicity); tetracyclines (increased risk of pseudotumor cerebri); coadministration with phenytoin increases toxicity (observe patient for signs of phenytoin toxicity and seizure control if coadministration absolutely necessary)
Documented hypersensitivity; pregnancy, breastfeeding, or women who intend to become pregnant within 3 y after discontinuing drug; impaired liver or kidney function; paraben sensitivity; psychiatric disturbance
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Common reactions include dry skin, cheilitis, photosensitivity, hypertriglyceridemia, hair loss, and decreased night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; diabetes patients may experience problems in controlling blood glucose; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occurs; caution in history of depression or other psychiatric disorders; associated with severe birth defects; females must use 2 forms of birth control throughout therapy, and pregnancy tests must be checked qmo; do not use in severe obesity; perform AST, ALT, and lipid tests prior to initiation of therapy, at 1- to 2-wk intervals until stable, and thereafter at intervals as clinically indicated
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.
Adult
0.5-2 mg/kg/d PO divided bid with food
Pediatric
>12 years: 0.5-2 mg/kg/d PO divided bid with food
Toxicity may occur with vitamin A or acitretin coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine; coadministration with MTX may cause liver toxicity (closely monitor for symptoms of liver toxicity if coadministration absolutely necessary)
Documented hypersensitivity, pregnancy, breastfeeding, depression, psychiatric disorders, inflammatory bowel disease, paraben sensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Common reactions include dry skin, cheilitis, photosensitivity, hypertriglyceridemia, hair loss, and decreased night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; diabetes patients may experience problems in controlling blood glucose; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occurs; caution in history of depression or other psychiatric disorders; associated with severe birth defects; female must use 2 forms of birth control throughout therapy and pregnancy tests must be checked qmo
Antimetabolites
These agents inhibit cell growth and proliferation. May also cause immunosuppression.24
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.
Adult
1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response; not to exceed 2.5 mg/kg/d
Pediatric
Administer as in adults
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of MTX metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine; alfalfa supplements may inhibit effects; inhibition by balsalazide of thiopurine methyltransferase; mercaptopurine may increase toxicity
Documented hypersensitivity; low levels of TPMT; pregnancy or breastfeeding; avoid live vaccines
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia and infection; caution with liver disease and renal impairment; hematologic toxicities may occur; adverse effects include bone marrow suppression, hypersensitivity, GI symptoms, or hepatotoxicity; if leukopenia occurs, consider checking TPMT levels (enzyme involved in metabolism of azathioprine); history of treatment with alkylating agents
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.
Adult
2.5-7.5 mg PO q12h for 3 doses/wk; alternatively, 10-25 mg/wk PO/IM; gradually increase dose, not to exceed 30 mg/wk; give with folic acid 1 mg qd or leucovorin 5 mg qwk
Pediatric
5-15 mg/m2/wk PO/IM/SC; may give divided dose q12h X 3; not to exceed 30 mg/m2/wk; give with folic acid 1 mg qd or leucovorin 5 mg qwk
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; folic acid or its derivatives contained in some vitamins may decrease response; probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides (including TMP-SMZ) can increase plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines; coadministration with hepatotoxic drugs may increase toxicity; concurrent administration with doxycycline, amoxicillin, and ciprofloxacin may increase toxicity of MTX; thiazide diuretics may enhance the myelosuppressive effects of antineoplastic agents
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBC counts monthly and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated levels [eg, dehydration]); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs; fatal reactions reported when administered concurrently with NSAIDs; may cause mucositis and skin ulceration
Immunosuppressants
These agents inhibit key factors that regulate the immune system. Case reports have shown benefit in some patients with pityriasis rubra pilaris.25
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.
Adult
2.5 mg/kg/d PO divided bid for 4 wk, then increase by 0.5 mg/kg/d q2wk until improvement; not to exceed 5 mg/kg/d
Pediatric
Not established
Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin; methylprednisolone and cyclosporine mutually inhibit one another resulting in increased plasma levels of each; coadministration with NSAIDs may result in increases in cyclosporine levels, nephrotoxicity, and increased plasma creatinine concentrations; concomitant use of ACE inhibitors with cyclosporine may decrease renal function
Concurrent use with potentially nephrotoxic medications such as amphotericin B or aminoglycosides may result in decreased renal function; coadministration of allopurinol or cimetidine may increase cyclosporine toxicity
Possible interaction with any medication that uses CYP3A4 hepatic enzymes for metabolism
Hypersensitivity, abnormal renal function, hypertension, malignancies, breastfeeding, live vaccines
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Associated with increased risk of infection and neoplasia; risk of hypertension, hyperkalemia, leukopenia, thrombocytopenia, nephrotoxicity, and hepatotoxicity; monitor CBC counts and liver and renal function regularly
Biologic agents
The use of monoclonal antibodies that suppress the immune system may improve the clinical aspects of the disease.26
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.
Adult
25 mg SC twice weekly or 50 mg SC once or twice weekly
Pediatric
Administer as in adults
None reported
Documented hypersensitivity; active infection; sepsis; concurrent live vaccination; demyelinating disorders or multiple sclerosis; tuberculosis
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in impaired renal function and asthma; discontinue administration if serious infection develops; adverse effects may include injection-site pain, localized erythema, rash, URI symptomology, GI upset, nausea, vomiting, rhinitis, cough, and drug-induced lupus; caution in congestive heart failure
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.
Adult
5 mg/kg IV infusion, followed with additional similar doses at 2 and 6 wk after first infusion, then q8wk thereafter
Pediatric
Not established
Increased risk of neutropenia and infection with coadministration of etanercept and anakinra
Hypersensitivity to murine proteins; patients with moderate to severe heart failure or active infections
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adverse effects include serious and potentially fatal infections, hepatotoxicity, heart failure, leukopenia, neutropenia, thrombocytopenia, pancytopenia, hypersensitivity reaction, neurologic events (optic neuritis, seizure, MS), increased risk of malignancy, and lupuslike syndrome; avoid live vaccines; avoid use in patients with active infection or concomitant immunosuppressive therapy
Vitamins
Agents like vitamin A have been reported to improve the clinical aspects of the disease.
Vitamin A
Reports of improvement with vitamin A; however, synthetic retinoids are more effective.
Adult
150,000-300,000 IU PO qd
Pediatric
Not established
May increase acitretin or isotretinoin toxicity; concomitant administration with anticoagulants or vitamin A can cause increased anticoagulant effect if given at large doses
Coadministration with tetracyclines and vitamin A may result in pseudotumor cerebri
Documented hypersensitivity; liver disease; renal disease
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Pregnancy category X in doses exceeding RDA; prolonged high doses may be hepatotoxic; nausea, vomiting, irritability, drowsiness, headache or coma may occur with higher doses
More on Pityriasis Rubra Pilaris |
| Overview: Pityriasis Rubra Pilaris |
| Differential Diagnoses & Workup: Pityriasis Rubra Pilaris |
 Treatment & Medication: Pityriasis Rubra Pilaris |
| Follow-up: Pityriasis Rubra Pilaris |
| Multimedia: Pityriasis Rubra Pilaris |
| References |
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References
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Further Reading
Keywords
pityriasis rubra pilaris, PRP, papulosquamous disorder, palmoplantar keratoderma, keratotic follicular papules, erythroderma
