The cause of pityriasis rubra pilaris is unknown.
The 2 most common forms of the disorder are
Juvenile classic (characterized by autosomal dominant inheritance and childhood onset)
Adult classic (characterized by no apparent inheritance and adult onset)
Atypical (nonclassic) forms exist in both age groups. Sunlight, HIV or another infection, minor trauma, or an autoimmune disorder may trigger a flare-up.
Pityriasis rubra pilaris can manifest as hyperkeratotic yellowing of the palms and soles.
Image provided by Thomas Habif, MD.
Pityriasis rubra pilaris can manifest as red follicular papules that coalesce to form red-orange scaling plaques and confluent areas of erythema with islands of normal skin between lesions.
Image provided by Thomas Habif, MD.
This image shows hyperkeratosis of the sole characteristic of pityriasis rubra pilaris.
Image courtesy of Karen McKoy, MD.
Diagnosis of Pityriasis Rubra Pilaris
Clinical evaluation
Sometimes biopsy
Diagnosis of pityriasis rubra pilaris is by clinical appearance and may be supported by biopsy.
Biopsy is done when the clinical diagnosis is unclear (eg, when the patient is erythrodermic at presentation).
Differential diagnosis of pityriasis rubra pilaris includes the following:
Treatment of Pityriasis Rubra Pilaris
Symptom relief (eg, with emollients, topical lactic acid and topical corticosteroids, or oral retinoids)
Sometimes systemic therapy (eg, oral acitretin, methotrexate, biologics)
Treatment of pityriasis rubra pilaris is exceedingly difficult and empiric. The disorder may be ameliorated but almost never cured; classic forms of the disorder resolve slowly over 3 years, whereas nonclassic forms persist.
Scaling may be reduced with emollients or 12% lactic acid under occlusive dressing, followed by topical corticosteroids.
Oral acitretin (a retinoid) or methotrexate is an option when the disorder is resistant to topical treatment. In addition, biologics such as etanercept (tumor necrosis factor inhibitor), ustekinumab (IL-12/IL-23 inhibitor), and secukinumab (IL-17 inhibitor) are being used increasingly based on limited observational data suggesting benefit and on indirect evidence of their efficacy for the treatment of psoriasis (1 Treatment references Pityriasis rubra pilaris is a rare chronic disorder that causes hyperkeratotic yellowing of the skin, including the trunk, extremities, and, particularly, the palms and soles. Red follicular... read more 
, 2 Treatment references Pityriasis rubra pilaris is a rare chronic disorder that causes hyperkeratotic yellowing of the skin, including the trunk, extremities, and, particularly, the palms and soles. Red follicular... read more ).
Phototherapy, oral vitamin A, cyclosporine, mycophenolate mofetil, azathioprine, and systemic corticosteroids have also been used (3 Treatment references Pityriasis rubra pilaris is a rare chronic disorder that causes hyperkeratotic yellowing of the skin, including the trunk, extremities, and, particularly, the palms and soles. Red follicular... read more ).
Treatment references
1. Eastham AB, Femia A, Qureshi A, et al: Treatment options for pityriasis rubra pilaris including biologic agents: A retrospective analysis from an academic medical center. JAMA Dermatol 150(1):92–94, 2014. doi: 10.1001/jamadermatol.2013.4773
2. Napolitano M, Abeni D, Didona B: Biologics for pityriasis rubra pilaris treatment: A review of the literature. J Am Acad Dermatol 79(2):353–359.e11, 2018. doi: 10.1016/j.jaad.2018.03.036
3. Engelmann C, Elsner P, Miguel D: Treatment of pityriasis rubra pilaris type I: A systematic review. Eur J Dermatol 29(5):524-537, 2019. doi: 10.1684/ejd.2019.3641
Drugs Mentioned In This Article
| Drug Name | Select Trade |
|---|---|
acitretin |
Soriatane |
methotrexate |
Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep |
etanercept |
Enbrel |
ustekinumab |
Stelara |
secukinumab |
Cosentyx |
vitamin a |
A Mulsin, Aquasol A, Dofsol-A |
cyclosporine |
Cequa, Gengraf , Neoral, Restasis, Sandimmune, SangCya, Verkazia |
mycophenolate |
CellCept, Myfortic |
azathioprine |
Azasan, Imuran |
