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Observation
November 2016

Successful Treatment of Refractory Pityriasis Rubra Pilaris With Secukinumab

Author Affiliations
  • 1Department of Dermatology, Medical Center–University of Freiburg, Freiburg, Germany
JAMA Dermatol. 2016;152(11):1278-1279. doi:10.1001/jamadermatol.2016.3885

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder of unknown cause. It is characterized by follicular hyperkeratosis, scaly erythematous plaques, palmoplantar keratoderma, and frequent progression to generalized erythroderma.1 Six types of PRP are distinguished, with type 1 being the most common form in adults. Disease management of PRP is challenging for lack of specific guidelines. Topical emollients, corticosteroids, and salicylic acid alone or combined with systemic retinoids, methotrexate, and tumor necrosis factor (TNF) inhibitors are considered to be most helpful.2,3 Unfortunately, PRP often resists conventional treatment. We report the case of a 67-year-old man with refractory PRP who was successfully treated with secukinumab.

Report of a Case

A 67-year-old man presented with an acute generalized, erythematous and scaly eruption with spared patches of unaffected skin on his chest (Figure 1A). He also had palmoplantar keratoderma, nail dystrophy, and severe pruritus. Skin biopsy confirmed the clinical diagnosis of PRP. Before onset of the PRP, he had not taken any new medication, and there was no evidence for allergies or chronic skin disorders in his personal or family history. Laboratory tests ruled out any infection, atopic disposition, autoimmune disease, or cancer. Treatment was started with acitretin, 35 mg/d, corresponding to 0.5 mg/kg of body weight. In addition, acute flares were treated with short-term systemic corticosteroid regimens combined with topical class IV corticosteroids.

Figure 1.  Clinical Images Before and After Secukinumab Treatment of Pityriasis Rubra Pilaris
Clinical Images Before and After Secukinumab Treatment of Pityriasis Rubra Pilaris

A, At presentation. B, After 8 weeks of treatment with secukinumab.

After 5 months of treatment, he still had severe pruritus and presented with erythroderma, scaling, and palmoplantar keratoderma. Because there were several contraindications for conventional immunosuppressive treatments, treatment was switched to secukinumab, a monoclonal anti–interleukin(IL)-17A antibody approved for the treatment of moderate to severe plaque psoriasis. After giving written informed consent, the patient received 2 subcutaneous injections of secukinumab, 150 mg each, once a week for 5 weeks, followed by monthly injections.

After 3 weeks of secukinumab treatment, the scaling and pruritus were clearly reduced, and after 8 weeks, pruritus and erythema had completely cleared (Figure 1B). Correspondingly, the typical histopathological features of PRP such as hyperplasia, acantholytic dyskeratosis, and hyperkeratosis (Figure 2A) had disappeared after 8 weeks of treatment with secukinumab (Figure 2B). After 6 months, the palmoplantar keratoderma had also disappeared, and nail growth was normal. No clinical or laboratory adverse effects were registered.

Figure 2.  Hematoxylin-Eosin–Stained Histopathological Images of Punch Biopsy Specimens From Pityriasis Rubra Pilaris (PRP) Lesions on the Back
Hematoxylin-Eosin–Stained Histopathological Images of Punch Biopsy Specimens From Pityriasis Rubra Pilaris (PRP) Lesions on the Back

A, The typical histopathological features of PRP are seen, such as hyperplasia, acantholytic dyskeratosis, and hyperkeratosis (original magnification ×400). B, These features had disappeared after 8 weeks of treatment with secukinumab (original magnification ×400).

Discussion

The treatment options for PRP are mainly based on clinical observations and are partly adopted from psoriasis therapy because psoriasis shares some clinical and histopathological features with PRP.4 In the last decade, various biological therapies have improved the treatment options of psoriasis. Some case reports have also reported successful treatment of PRP with TNF and IL-12/23 antagonists.3,5 We demonstrate for the first time to our knowledge that the IL-17A inhibitor secukinumab is effective in the treatment of severe, therapy-refractory PRP. Secukinumab was approved for the treatment of moderate to severe plaque psoriasis in 2015.6 In the present patient with PRP, skin alterations almost completely disappeared after 6 months of treatment. We cannot completely exclude the possibility that, at least in part, spontaneous remission of PRP occurred because up to 80% of type 1 PRP type 1 is self-limited without treatment within 3 years after onset.2 However, we started treatment in a phase of severe, generalized PRP with acute exacerbations, and spontaneous healing is unlikely in this case. Further studies are required to evaluate the efficacy and safety of IL-17 antagonists in larger cohorts of patients with PRP.

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Article Information

Corresponding Author: Christoph M. Schempp, MD, PhD, Department of Dermatology, Medical Center–University of Freiburg, Hauptstr 7, D-79104 Freiburg, Germany (christoph.schempp@uniklinik-freiburg.de).

Correction: This article was corrected on November 23, 2016, to add omitted third author’s name in print versions.

Published Online: October 5, 2016. doi:10.1001/jamadermatol.2016.3885

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Albert  MR, Mackool  BT.  Pityriasis rubra pilaris.  Int J Dermatol. 1999;38(1):1-11.PubMedGoogle ScholarCrossref
2.
Ross  NA, Chung  HJ, Li  Q, Andrews  JP, Keller  MS, Uitto  J.  Epidemiologic, clinicopathologic, diagnostic, and management challenges of pityriasis rubra pilaris: a case series of 100 patients.  JAMA Dermatol. 2016;152(6):670-675.PubMedGoogle ScholarCrossref
3.
Petrof  G, Almaani  N, Archer  CB, Griffiths  WA, Smith  CH.  A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF-antagonists.  J Eur Acad Dermatol Venereol. 2013;27(1):e131-e135.PubMedGoogle ScholarCrossref
4.
Magro  CM, Crowson  AN.  The clinical and histomorphological features of pityriasis rubra pilaris: a comparative analysis with psoriasis.  J Cutan Pathol. 1997;24(7):416-424.PubMedGoogle ScholarCrossref
5.
Chowdhary  M, Davila  U, Cohen  DJ.  Ustekinumab as an alternative treatment option for chronic pityriasis rubra pilaris.  Case Rep Dermatol. 2015;7(1):46-50.PubMedGoogle ScholarCrossref
6.
Strober  B, Sigurgeirsson  B, Popp  G,  et al.  Secukinumab improves patient-reported psoriasis symptoms of itching, pain, and scaling: results of two phase 3, randomized, placebo-controlled clinical trials.  Int J Dermatol. 2016;55(4):401-407.PubMedGoogle ScholarCrossref
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