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Pityriasis Rubra Pilaris

Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Pityriasis rubra pilaris (PRP) is a rare papulosquamous inflammatory skin condition characterized by red-orange scaly plaques, palmoplantar keratoderma, and follicular keratotic papu...

DIAGNOSIS

Studies show only 1/4 of patients are correctly diagnosed at initial presentation. 
  • Initial missed diagnoses include psoriasis, contact dermatitis, eczema/spongiotic dermatitis, seborrheic de...

TREATMENT

  • No published randomized controlled trials exist due to the rarity of this disorder.

  • Topical therapies have limited effectiveness when used as sole therapy and are often used in combination wi...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Patients who develop erythroderma should be monitored for electrolyte abnormalities, hypoalbuminemia, hypothermia, secondary bacterial infection...

REFERENCES

1
Petrof G, Almaani N, Archer CB, et al. A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF-antagonists. J Eur Acad Dermatol Venereol.  2...

ADDITIONAL READING

  • Amann PM, Susic M, Glüder F, et al. Alitretinoin (9-cis retinoic acid) is effective against pityriasis rubra pilaris: a retrospective clinical study. Acta Derm Venereol.  2015;...

CODES

ICD10

L44.0 Pityriasis rubra pilaris 

ICD9

696.4 Pityriasis rubra pilaris 

SNOMED

  • 3755001 Pityriasis rubra pilaris (disorder)

  • 238618003 Classical adult pityriasis rubra pilaris

  • 238619006 Atypical adult...

CLINICAL PEARLS

  • PRP is a rare chronic skin condition with an unknown etiology that can have a profound negative impact on patients’ self-image. Depression is often present, with only 12% of patients i...

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