eMedicine Specialties > Dermatology > Papulosquamous Diseases

Pityriasis Rubra Pilaris

Philip D Shenefelt, MD, MS, Associate Professor, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine; Past Chief, Section of Dermatology, James A Haley Veteran Affairs Medical Center

Updated: May 22, 2009

Introduction

Background

Pityriasis rubra pilaris (PRP) was first described in 1828 by Tarral and was named by Besnier in 1889. It is a chronic papulosquamous disorder of unknown etiology characterized by reddish orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. The disease may progress to erythroderma with distinct areas of uninvolved skin, the so-called islands of sparing.

Griffiths divided pityriasis rubra pilaris into 5 categories: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, and atypical juvenile type.^1,2 More recently, an HIV-associated type has been added to this classification system.^3,4,5,6

A few reports have also described pityriasis rubra pilaris associated with underlying malignancy.⁷

Other eMedicine pityriasis articles include Pityriasis Alba, Pityriasis Lichenoides, Pityriasis Rosea, and Pityriasis Rotunda.

Pathophysiology

The etiology is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern; however, most cases are sporadic.⁸ One hypothesis is that pityriasis rubra pilaris may be related to an abnormal immune response to an antigenic trigger. Case reports have described pityriasis rubra pilaris occurring after streptococcal infections.⁹

Frequency

United States

The incidence of pityriasis rubra pilaris has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics.

Mortality/Morbidity

Patients with pityriasis rubra pilaris can have painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may be present. However, most of the morbidity associated with pityriasis rubra pilaris is associated with the erythroderma (see Complications).

Race

Persons of any race can be affected.

Sex

Pityriasis rubra pilaris occurs equally among men and women.¹⁰

Age

• The familial form of pityriasis rubra pilaris typically begins in early childhood and has an autosomal dominant inheritance pattern.
• The acquired form of pityriasis rubra pilaris has a bimodal age distribution, with peaks in the first and fifth decades of life, but it can begin at any age.

Clinical

History

• The familial form of pityriasis rubra pilaris has a gradual onset, whereas the acquired form has an acute onset.
• The disease typically spreads in a craniocaudal direction.
  • Patients first notice redness and scales on the face and the scalp.
  • This is often followed by redness and thickening of the palms and the soles (see Media Files 3-4).

Plantar keratoderma with an orange hue on the soles.

Palmar keratoderma with an orange hue on the palms.

• The lesions may expand and coalesce to cover the entire body.

Physical

• Skin
  • Pityriasis rubra pilaris is characterized by orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body (see Media File 1).

Reddish orange plaques on the trunk.

• Often, areas of uninvolved skin, referred to as islands of sparing, are present.
• Follicular hyperkeratosis is commonly seen on the dorsal aspects of the proximal phalanges, the elbows, and the wrists (see Media File 2). This pattern may be referred to as nutmeg grater papules.

Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.

• • Palmoplantar keratoderma occurs in most patients and tends to have an orange hue. Painful fissures may develop in patients with palmoplantar keratoderma.
  • Pruritus, although not a major symptom, may occur in the early stages of the disease.
• Nails
  • Nail changes include distal yellow-brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages.
  • Nail pitting is not typical.
• Mucous membranes: Patients may complain of pain and irritation in the mouth. Mucous membrane changes include a diffuse whitish appearance of the buccal mucosa, lacy whitish plaques, grayish-white papules and plaques, erythema, or possible erosions.¹¹
• Eyes: Patients with extensive disease may develop ectropion. Patients have also reported blurred vision and dryness.
• Griffiths classification
  • Type I is classic adult pityriasis rubra pilaris. This is the most common form of pityriasis rubra pilaris, accounting for more than 50% of all cases of pityriasis rubra pilaris. Onset is acute, and the features are classic, including erythroderma with islands of sparing, palmoplantar keratoderma, and follicular hyperkeratosis. This type of pityriasis rubra pilaris has the best prognosis. Reportedly, about 80% of patients have remission in an average of 3 years.
  • Type II is atypical adult pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. It is characterized by ichthyosiform lesions, areas of eczematous change, alopecia, and long duration (often 20 y or more.)
  • Type III is classic juvenile pityriasis rubra pilaris. This form accounts for about 10% of all cases of pityriasis rubra pilaris. It is very similar to type I; however, its onset is within the first 2 years of life. Remission can occur sooner than with type I, within an average of 1 year.
  • Type IV is circumscribed juvenile pityriasis rubra pilaris. This form accounts for about 25% of all cases of pityriasis rubra pilaris. It occurs in prepubertal children and is characterized by sharply demarcated areas of follicular hyperkeratosis and erythema of the knees and the elbows. The long-term outcome is unclear, with some reports of improvement in the late teenaged years. This form of pityriasis rubra pilaris rarely progresses.
  • Type V is atypical juvenile pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. Most cases of familial pityriasis rubra pilaris belong to this group. It has an early onset and runs a chronic course. It is characterized by prominent follicular hyperkeratosis, sclerodermalike changes on the palms and the soles, and infrequent erythema.
  • Type VI is HIV-associated pityriasis rubra pilaris. Patients with HIV may have nodulocystic and pustular acneiform lesions. Elongated follicular plugs or lichen spinulosus–type lesions have also been reported to be present. Patients' conditions tend to be resistant to standard treatments, but they may respond to antiretroviral therapies.

Causes

The etiology is unknown.

Differential Diagnoses

Cutaneous T-Cell Lymphoma
Erythroderma (Generalized Exfoliative Dermatitis)
Erythrokeratodermia Variabilis
Psoriasis, Plaque

Workup

Laboratory Studies

• No specific laboratory tests are available to confirm the diagnosis of pityriasis rubra pilaris (PRP). The diagnosis is usually made on the basis of a correlation between clinical findings and histologic findings.¹²
• Perform tests for electrolyte abnormalities, hypoalbuminemia, secondary bacterial infection in the skin, and possible sepsis.

Procedures

• Perform a biopsy.

Histologic Findings

Histologic features are not pathognomonic, but they are useful to rule out other possible papulosquamous and erythrodermic disorders. Features on light microscopy include hyperkeratosis with alternating orthokeratosis and parakeratosis forming a checkerboard pattern in the stratum corneum, focal or confluent hypergranulosis, follicular plugging with perifollicular parakeratosis forming a shoulder effect, thick suprapapillary plates, broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic perivascular infiltration. Acantholysis has been reported as an additional histologic finding in pityriasis rubra pilaris.¹³ Acantholysis may be restricted to adnexal epithelium. The presence of acantholysis, hypergranulosis, follicular plugging, and the absence of dilated capillaries and epidermal pustulation may help distinguish pityriasis rubra pilaris from psoriasis.¹⁴

Features on electron microscopy include a decreased number of keratin filaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipidlike vacuoles, large numbers of lamellar granules, and a focal split in the basal lamina at the dermoepidermal junction.

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.

Dosing

Adult

Recommended dose: 25-50 mg PO qd; take with food

Pediatric

Not established

Interactions

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)

Contraindications

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

Precautions

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.

Dosing

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

Interactions

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)

Contraindications

Documented hypersensitivity, pregnancy, breastfeeding, depression, psychiatric disorders, inflammatory bowel disease, paraben sensitivity

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; low levels of TPMT; pregnancy or breastfeeding; avoid live vaccines

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Hypersensitivity, abnormal renal function, hypertension, malignancies, breastfeeding, live vaccines

Precautions

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.

Dosing

Adult

25 mg SC twice weekly or 50 mg SC once or twice weekly

Pediatric

Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity; active infection; sepsis; concurrent live vaccination; demyelinating disorders or multiple sclerosis; tuberculosis

Precautions

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.

Dosing

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

Interactions

Increased risk of neutropenia and infection with coadministration of etanercept and anakinra

Contraindications

Hypersensitivity to murine proteins; patients with moderate to severe heart failure or active infections

Precautions

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.

Dosing

Adult

150,000-300,000 IU PO qd

Pediatric

Not established

Interactions

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

Contraindications

Documented hypersensitivity; liver disease; renal disease

Precautions

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

Follow-up

Further Outpatient Care

• Pityriasis rubra pilaris (PRP) patients who have erythroderma should be monitored for electrolyte abnormalities, hypoalbuminemia, secondary bacterial infection in the skin, and possible sepsis.

Complications

• Pityriasis rubra pilaris can cause painful and disabling palmoplantar keratoderma.
• Nail dystrophy and shedding may occur.
• Erythroderma is a reaction pattern of the skin that can occur in the setting of several different skin disorders, most commonly including psoriasis, eczema, lymphoma, drug reactions, and pityriasis rubra pilaris. It is characterized by generalized erythema and scales, hair loss, and onycholysis.
  • Systemic symptoms include malaise, fatigue, anorexia, fever, and chills.
  • Patients with erythroderma may develop lymphadenopathy, hepatomegaly, splenomegaly, and electrolyte abnormalities due to increased transepidermal water loss. Cardiac failure may occur in patients with preexisting heart conditions.

Prognosis

• Each type of pityriasis rubra pilaris has its own prognosis. In general, the familial form of the disease may be persistent throughout life, and the acquired form of the disease may resolve spontaneously within 1-3 years.

Patient Education

• Greene reported on pityriasis rubra pilaris support groups.²⁷

Miscellaneous

Medicolegal Pitfalls

• If pityriasis rubra pilaris appears in an older patient or if it seems atypical, the patient should be evaluated for a possible underlying malignancy.

Multimedia

Media file 1: Reddish orange plaques on the trunk.

Media file 2: Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.

Media file 3: Plantar keratoderma with an orange hue on the soles.

Media file 4: Palmar keratoderma with an orange hue on the palms.

References

1. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. Mar 1980;5(1):105-12. [Medline].

2. Griffiths WA. Pityriasis rubra pilaris: the problem of its classification. J Am Acad Dermatol. Jan 1992;26(1):140-2. [Medline].

3. Auffret N, Quint L, Domart P, Dubertret L, Lecam JY, Binet O. Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. J Am Acad Dermatol. Aug 1992;27(2 Pt 1):260-1. [Medline].

4. Blauvelt A, Nahass GT, Pardo RJ, Kerdel FA. Pityriasis rubra pilaris and HIV infection. J Am Acad Dermatol. May 1991;24(5 Pt 1):703-5. [Medline].

5. Martin AG, Weaver CC, Cockerell CJ, Berger TG. Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne. Br J Dermatol. Jun 1992;126(6):617-20. [Medline].

6. Miralles ES, Nunez M, De Las Heras ME, Perez B, Moreno R, Ledo A. Pityriasis rubra pilaris and human immunodeficiency virus infection. Br J Dermatol. Dec 1995;133(6):990-3. [Medline].

7. Kurzydlo AM, Gillespie R. Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma. Australas J Dermatol. May 2004;45(2):130-2. [Medline].

8. Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P. Familial pityriasis rubra pilaris. Arch Dermatol. Apr 1995;131(4):448-53. [Medline].

9. Mohrenschlager M, Abeck D. Further clinical evidence for involvement of bacterial superantigens in juvenile pityriasis rubra pilaris (PRP): report of two new cases. Pediatr Dermatol. Nov-Dec 2002;19(6):569. [Medline].

10. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol. Sep 2002;47(3):386-9. [Medline].

11. Martinez Calixto LE, Suresh L, Matsumura E, Aguirre A, Radfar L. Oral pityriasis rubra pilaris. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. May 2006;101(5):604-7. [Medline].

12. Cohen PR, Prystowsky JH. Pityriasis rubra pilaris: a review of diagnosis and treatment. J Am Acad Dermatol. May 1989;20(5 Pt 1):801-7. [Medline].

13. Howe K, Foresman P, Griffin T, Johnson W. Pityriasis rubra pilaris with acantholysis. J Cutan Pathol. Jun 1996;23(3):270-4. [Medline].

14. Magro CM, Crowson AN. The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. J Cutan Pathol. Aug 1997;24(7):416-24. [Medline].

15. Van de Kerkhof PC, Steijlen PM. Topical treatment of pityriasis rubra pilaris with calcipotriol. Br J Dermatol. May 1994;130(5):675-8. [Medline].

16. Karimian-Teherani D, Parissa M, Tanew A. Response of juvenile circumscribed pityriasis rubra pilaris to topical tazarotene treatment. Pediatr Dermatol. Jan-Feb 2008;25(1):125-6. [Medline].

17. Haenssle HA, Bertsch HP, Emmert S, Wolf C, Zutt M. Extracorporeal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris. Clin Exp Dermatol. May 2004;29(3):244-6. [Medline].

18. Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. Int J Dermatol. Apr 2006;45(4):438-46. [Medline].

19. Cox V, Lesesky EB, Garcia BD, O'Grady TC. Treatment of juvenile pityriasis rubra pilaris with etanercept. J Am Acad Dermatol. Nov 2008;59(5 Suppl):S113-4. [Medline].

20. Liao WC, Mutasim DF. Infliximab for the treatment of adult-onset pityriasis rubra pilaris. Arch Dermatol. Apr 2005;141(4):423-5. [Medline].

21. Manoharan S, White S, Gumparthy K. Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab. Australas J Dermatol. May 2006;47(2):124-9. [Medline].

22. Muller H, Gattringer C, Zelger B, Hopfl R, Eisendle K. Infliximab monotherapy as first-line treatment for adult-onset pityriasis rubra pilaris: case report and review of the literature on biologic therapy. J Am Acad Dermatol. Nov 2008;59(5 Suppl):S65-70. [Medline].

23. Dicken CH. Treatment of classic pityriasis rubra pilaris. J Am Acad Dermatol. Dec 1994;31(6):997-9. [Medline].

24. Clayton BD, Jorizzo JL, Hitchcock MG, et al. Adult pityriasis rubra pilaris: a 10-year case series. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):959-64. [Medline].

25. Wetzig T, Sticherling M. Juvenile pityriasis rubra pilaris: successful treatment with ciclosporin. Br J Dermatol. Jul 2003;149(1):202-3. [Medline].

26. Borok M, Lowe NJ. Pityriasis rubra pilaris. Further observations of systemic retinoid therapy. J Am Acad Dermatol. May 1990;22(5 Pt 1):792-5. [Medline].

27. Greene R. PRP support group. Dermatol Nurs. Feb 2006;18(1):28. [Medline].

28. Griffiths WA. Pityriasis rubra pilaris--an historical approach. 2. Clinical features. Clin Exp Dermatol. Mar 1976;1(1):37-50. [Medline].

Keywords

pityriasis rubra pilaris, PRP, papulosquamous disorder, palmoplantar keratoderma, keratotic follicular papules, erythroderma

Contributor Information and Disclosures

Author

Philip D Shenefelt, MD, MS, Associate Professor, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine; Past Chief, Section of Dermatology, James A Haley Veteran Affairs Medical Center
Philip D Shenefelt, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Physician Executives, American Contact Dermatitis Society, American Medical Association, American Society of Clinical Hypnosis, Florida Medical Association, Noah Worcester Dermatological Society, and Society for Clinical and Experimental Hypnosis
Disclosure: Nothing to disclose.

Medical Editor

Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Abbott Laboratories Honoraria Consulting; Actelion Honoraria Consulting; Amgen Honoraria Consulting; Astellas Honoraria Consulting; Centocor Honoraria Consulting; DermiPsor Honoraria Consulting; Galderma  Consulting; Genentech Honoraria Consulting; Helix BioMedix Honoraria Consulting; Medicis Honoraria Investigator

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Margaret H. Rinker, MD, to the development and writing of this article.

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