Treatment Options for Pityriasis Rubra Pilaris Including Biologic Agents: A Retrospective Analysis From an Academic Medical Center

Pityriasis rubra pilaris (PRP) is characterized by hyperkeratotic papules, palmoplantar keratoderma, and widespread erythema with islands of sparing. Treatment is challenging, and a standard therapeutic protocol does not exist. Given the paucity of available therapeutic data, we assessed the clinical presentation of PRP and clinical response (CR) to therapy at 2 teaching hospitals in Boston, Massachusetts. In addition, we aimed to determine the role of systemic and biologic agents.

Using the university-based Partners Healthcare Research Patient Data Registry, we identified all patients with PRP seen by a dermatologist from January 2000 through December 2011. The database query was based on a natural language search of “PRP,” “International Classification of Diseases, Ninth Revision code 696.4,” and “dermatology providers.” We extracted data on disease presentation, treatment, and CR. Response was defined as no CR, partial CR (<75% improvement), or marked CR (≥75% improvement) from baseline. The Brigham and Women’s Hospital institutional review board approved this study.

Of 176 patients identified, 40 had a diagnosis of PRP, 38 of whom received this diagnosis based on clinical and pathological findings. There were 36 patients with adult type 1 PRP (of whom 3 had associated polyarthritis and 6 required hospitalization), 3 with juvenile type 4, and 1 with juvenile type 5. There was no sex predominance, and the majority of patients were white. The mean age of adult and childhood onset was 57 and 5 years, respectively, with a 2-year mean disease duration.

Partial to marked CR occurred in 14 patients given topical corticosteroid monotherapy. Narrowband UV-B, UV-A1, and bath psoralen plus UV-A provided partial CR in 3 patients. One patient received standard psoralen plus UV-A therapy without benefit. Twenty-five patients received acitretin, methotrexate, methotrexate plus acitretin, mycophenolate mofetil, or cyclosporine (Table 1 and Table 2). Ten patients received biologic agents, which were frequently used concomitantly with additional therapy (Table 2). Two patients had no CR to alefacept, whereas all patients treated with tumor necrosis factor (TNF) antagonists had partial to marked CR. The mean interval between initiation of TNF inhibition and notable CR was 5.7 weeks.

Pityriasis rubra pilaris presents a therapeutic challenge. Traditionally, treatment consisted of topical corticosteroids, acitretin, methotrexate, cyclosporine, and phototherapy. Within the last decade, several case reports have noted efficacy of TNF inhibition. The remaining literature on anti-TNF therapy is limited to 1 case series of 7 patients, all of whom demonstrated CR, and 1 retrospective review, featuring 1 patient with CR to infliximab.^1,2 Successful treatment with infliximab monotherapy as first-line treatment has been reported in 1 case.³ To our knowledge, this work is the largest review to date including outcomes of biologic therapy for PRP.

A recent study demonstrated upregulation of TNF mRNA in lesional compared with nonlesional skin in 2 patients with PRP.⁴ The degree of mRNA upregulation was comparable to that of 1 control patient with psoriasis.⁴ These data, along with our finding of marked CR in more than 50% of patients treated with TNF antagonists, suggest that TNF may be a key mediator in the pathophysiologic mechanism of PRP.

Rheumatologic associations with PRP are not well defined. To date, there have been 10 reported cases of arthritis occurring concomitantly with PRP.⁵ Within our patient population, 3 developed polyarthritis concomitant with their cutaneous disease. Of these, 1 had marked CR to acitretin, whereas 2 required TNF antagonists. Each had a prolonged disease course ranging from 6 to 14 years.

Limitations of our study include its retrospective nature, small sample size, and the possibility of spontaneous resolution as opposed to therapeutic effect. These data indicate that therapeutic response in PRP varies greatly, with some patients responding to topical therapy and others requiring systemic immunosuppression. Furthermore, polyarthritis may portend chronic disease and the necessity for systemic agents. Additional systematic investigation is necessary to determine which patients with PRP may respond to individual therapeutic options.

Corresponding Author: A. Brooke Eastham, MD, Harvard Combined Dermatology Program and Department of Dermatology, Brigham and Women’s Hospital, 221 Longwood Ave, Boston, MA 02115 (aeastham@partners.org).

Published Online: August 28, 2013. doi:10.1001/jamadermatol.2013.4773.

Author Contributions: All authors had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: Eastham, Femia, Vleugels.

Acquisition of data: Eastham, Femia.

Analysis and interpretation of data: Eastham, Femia, Qureshi, Vleugels.

Drafting of the manuscript: Eastham, Femia, Qureshi, Vleugels.

Critical revision of the manuscript for important intellectual content: Femia, Qureshi, Vleugels.

Statistical analysis: Qureshi.

Study supervision: Femia, Qureshi, Vleugels.

Conflict of Interest Disclosures: Dr Qureshi has been a consultant for Abbvie, the Centers for Disease Control and Prevention, Janssen, Novartis, and Pfizer, has received a grant from Amgen, and has received royalties from licensure via Brigham and Women's Hospital of outcomes tools to Janssen, Merck, and Pfizer. No other disclosures are reported.

Funding/Support: Dr Vleugels' career has been supported by a Medical Dermatology Career Development Award from the Dermatology Foundation.