PITYRIASIS RUBRA PILARIS

By Gary M. White, MD

Pityriasis rubra pilaris Thickening and orangish yellow discoloration of the palms.


Pityriasis rubra pilaris (PRP) is a relatively uncommon inflammatory disorder which is characterized by hyperkeratotic follicular papules, shiny orangish hyperkeratotic palms, and sharply demarcated islands of spared skin. See also PRP, Juvenile.

Clinical

PRP classically presents with orangish red follicular papules and coalescent plaques. The diagnosis is often suggested by the characteristic appearance of the palms which often take on a thickened orange hyperkeratosis that is said to resemble carnuba wax. Some adults have disease which rapidly progresses to total body erythroderma. The dorsa of the hands and fingers may be affected by follicular plugging. In contrast to psoriasis, nail pits are absent. Often, the condition starts on the head and develops "downward". Another classic sign is islands of sparing (or normal skin). PRP often spontaneously resolves after 1-3 years.

PRP has been divide into 6 subtypes (see Dermatology Online Journal 11(4)).

Type Comments
I Adult, Classic
II Adult, Atypical
III Juvenile, Classic
IV Juvenile, Circumscribed
V Juvenile, Atypical
VI HIV-Associated

Treatment-Adults

Aggressive treatment may not be indicated for mild disease. Topical emollients may be soothing. Topical steroids and calcipotriol may be given for limited disease. For widespread PRP an oral retinoid e.g. acitretin 25-50 QD or isotretinoin is usually given. It may take several weeks to a month or so to have its effect. If one retinoid does not work, sometimes the other will. If retinoids fail, the addition of weekly methotrexate therapy may be helpful.

Biologic agents have recently been employed with good results including ustekinumab, secukinumab, adalimumab, etanercept and infliximab. One patient responded within two months to monotherapy with 90 mg ustekinumab [Case Rep Dermatol 2015;7;46]. One of my patients who failed acitretin 75 mg/day as well as adalimumab cleared completely after two injections of ustekinumab 40 mg separated by a month.

Other therapies reported helpful include: cyclosporin (e.g. 5 mg/kg/d) [AD 1993;129;1346] and alitretinoin [Case Rep Dermatol 2011;3:85–88]. In one review, alitretinoin was highly effective in the treatment of PRP in 4 of 5 cases [Acta Dermato-Venereologica 2015;95;329]. Extracorporeal photochemotherapy has been recommended for erythrodermic PRP [AD 1999;135;475]. Acitretin has been combined with narrow-band UVB with benefit in a 12 year old girl [BJD 2000;142;370]. After 25 treatments, the PRP had completely resolved in the exposed areas. Acitretin plus UVA1 was helpful in one patient. One patient with refractory PRP responded with near complete resolution after 6-8 months of apremilast [JAMA Dermatol 2016;152;348].

Combination therapy is the rule with resistant disease. For example, one patient was treated with the combination of infliximab 5-7.5 mg/kg, MTX 12.5 mg/wk and acitretin 25/day (JAMA Derm Jan 2014;92).

Patients may need to get their ears cleaned out 3-4/year. Ophthalmology may be needed for ectropion and symblepharon.

Additional Pictures

The classics of PRP: orange/red rash with islands of sparing, plus the characteristic palms.
Pityriasis rubra pilaris Pityriasis rubra pilaris

Islands of sparing.
Pityriasis rubra pilaris Pityriasis rubra pilaris

Pityriasis rubra pilaris Pityriasis rubra pilaris with islands of sparing

Pityriasis rubra pilaris Pityriasis rubra pilaris

At times, PRP has a rippled scale pattern.
Pityriasis rubra pilaris pityriasis rubra pilaris

References

Rarely gyrate lesions may form in pityriasis rubra pilaris. JAAD 2013;69;e32–e33.

PRP in this report appeared to be paraneoplastic as it cleared within a week after the removal of the small cell carcinoma of the lung. Dermatology Online Journal 17(11)

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