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Chapter 7: Pityriasis Rubra Pilaris

Silvia Mihalceanu; Knut Schäkel

PITYRIASIS RUBRA PILARIS

Therapeutic Approach

Pityriasis rubra pilaris (PRP) is an inflammatory papulosquamous dermatosis of juvenile or adult onset. It is presenting with distinctive clinical features and a self-limiting or chronic evolution. The disease is subclassified into 6 types, including both hereditary and acquired forms. The hereditary form of PRP is linked to CARD14 gain-of-function mutations. The cause of the acquired forms I-IV is unknown. Type VI PRP is associated with HIV infection. Typical features are follicular hyperkeratotic papules that coalesce into scaly, reddish-orange–colored plaques. The disease may progress to erythroderma with well-demarcated islands of normal skin (“nappes claires”). Distinguishing PRP from psoriasis may pose a diagnostic challenge, particularly in early phases of the disease. Histopathologic examination reveals hyperkeratosis, alternating parakeratosis and orthokeratosis in a checkerboard pattern, with focal acantholytic dyskeratosis. The most successful treatment options are systemic retinoids, methotrexate as well as biologics; however, there is currently no FDA-approved therapy for PRP. Combining different systemic treatments or adjuvant photochemotherapy may be considered. See Table 7-1.

Table 7-1Pityriasis Rubra Pilaris Treatment Table
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Table 7-1 Pityriasis Rubra Pilaris Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Gycerin, Lanolin, Paraffin, Urea 5% Adjuvant therapy PRP type I-VI Emollients Apply QD-BID None None IV1-5

Salicylic acid 0.5-5%

Urea 10-40%

 Adjuvant therapy PRP type I-VI Keratolytics Apply QD Skin irritation None IV4-7
Vitamin D3 (Calcipotriol) PRP type I-VI Anti-inflammatory; inhibition of epidermal proliferation

Calcipotriol 0.005% ointment/cream

<30% of the body surface

Apply QD

Skin irritation

Hyper-/hypo-pigmentation

Photosensitivity

Hypercalcemia

Hypercalciuria

 None IV1,4-6,8,9
Topical glucocorticoids (eg, Mometasone, Betametasone, Prednicarbat) PRP type I-VI Anti-inflammatory NF-kB regulator

Mometasone 0.1% cream

Betamethasone

0.1% ointment/cream/lotion

Prednicarbat 0.25% cream/ointment

Apply QD

 Skin atrophy None III1,2,4-7,10
Calcineurine inhibitors (Tacrolimus Pimecrolimus) PRP type I-VI Anti-inflammatory

Tacrolimus 0.1% ointment

Pimecrolimus 1% cream

Apply QD

 Skin irritation None IV4-6,11
Systemic Therapy

Retinoids

Isotretinoin

Acitretin

Alitretinoin

Etretinate

 First-line therapy PRP type I-V Anti-inflammatory; inhibition of keratinocyte proliferation

Isotretinoin 0.5-2 mg/kg/d, washout 5 wk

Acitretin 0.5-0.75 mg/kg/d, washout 3 y

Alitretinoin 30 or 10 mg/d

Etretinate starting dose 10-20 mg/d, maintenance regimen 25-50 mg/d

Teratogenicity, skin dryness, nausea, increased lipids and liver enzymes; photosensitivity

Premature closes of epiphyses or hyperostosis

Temporary hair loss

Pregnancy test and contraception 1 mo before treatment, monthly and for a further 1 mo (Isotretinoin, Alitretinoin), 3 y (Acitretin) after discontinuation of therapy

CBC, CMP before treatment, every 2-4 wk for first 2 mo, then every 3 mo

Lipid panel before treatment, after 4 wk, then every 3 mo

 III2,4,12-18
Methotrexate Second-line therapy PRP type I-V Inhibition of DHF-reductase MTX 10-25 mg SC weekly + Folic acid 5 mg after 24 h

Teratogenicity

Liver enzyme elevation

Nausea, dizziness, vomiting

...

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