Pityriasis Rubra Pilaris Treatment & Management

Updated: Sep 11, 2020
  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Topical therapy

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. [22]

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. [23]

Emollients reduce fissuring and dryness, providing some patient comfort. Petroleum jelly or one of the many proprietary emollients may be used.

Biologics targeting tumor necrosis factor-alpha, interleukins 12 and 23, and Th17 can produce remissions. [24, 25, 26, 27] Those targeting interleukins 12 and 23 are particularly helpful in early-onset disease related to CARD14 mutation. [28, 29, 30, 31, 32, 33, 34]

Phototherapy

Phototherapy can include narrowband phototherapy and extracorporeal photochemotherapy.

Nonresponsiveness to treatment with topical and systemic medications should prompt consideration of narrowband UVB phototherapy. Narrowband UVB phototherapy uses a fluorescent bulb with a narrow emission spectrum that peaks at 311 nm (UVB spectrum, 290-320 nm). This selective and relatively longer wavelength may be more effective than broadband UVB for the treatment of pityriasis rubra pilaris. [35]

Extracorporeal photochemotherapy 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. [36] Further studies are needed.

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Long-Term Monitoring

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

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