Remission of Pityriasis Rubra Pilaris with IL-17A Antagonist Secukinumab

Case Report

Austin J Dermatolog. 2016; 3(5): 1063.

Remission of Pityriasis Rubra Pilaris with IL-17A Antagonist Secukinumab

Mariasy, Campanelli and Sauder DN*

Yardley Dermatology Associates, USA

*Corresponding author: Sauder DN, Yardley Dermatology Associates, 903 Floral Vale Blvd, Yardley, PA, 19067, USA

Received: October 12, 2016; Accepted: November 23, 2016; Published: November 25, 2016


Pityriasis Rubra Pilaris (PRP) is a rare inflammatory disease of unknown etiology associated with follicular hyperkeratosis, scaly erythematous plaques, palmoplantar keratoderma and generalized erythroderma [1]. Treatment of PRP is difficult as there is no standard protocol. Traditional treatments used have included oral retinoids, methotrexate, TNF-a inhibitors and corticosteroids. After failing the above therapies, we then attempted to control this particular case with the oral phosphodiesterase-4 inhibitor (PDE4) Otezla without success. We report of a case of remission of Pityriasis Rubra Pilaris with the administration of the newly marketed interleukin-17A antagonist Secukinumab (Cosentyx, Novartis). These findings suggest a possible correlation between interleukin- 17a and this particular papulosquamous eruption.

Keywords: Pityriasis rubra pilaris; Erythroderma; Cytokines


PRP: Pityriasis Rubra Pilaris; IL-17A: Interleukin 17A; Nb-UVB: Narrowband Ultraviolet B; MTX: Methotrexate


Pityriasis Rubra Pilaris (PRP) is a rare skin disorder which can affect both adults and children. While current research is suggesting a genetic component in familial PRP with the mutation of CARD14 [2], acute adult onset PRP in patients over the age of fifty is the more common presentation of the disease. Treatment for PRP can prove difficult, and common first line therapies include oral retinoids, methotrexate, TNF-a inhibitors and UVB narrowband therapy [3]. More recently, cases have been reported achieving remission with non-traditional therapeutic methods such as the Phosphodiesterase 4 (PDE4) Inhibitor, apremilast (Otezla; Celgene) [4], though it yielded no improvement for our patient. We report our findings of remission with secukinumab to potentially suggest a connection amongst IL- 17A and the pathophysiological mechanism of PRP.

Case Presentation

A 68-year-old white male presented in office complaining of an itchy rash that had been present for several weeks. Clinically, he presented with erythematous scaling with patches on the neck, forehead and torso. Our initial clinical differential diagnosis included pityriasis lichenoides chronica, pityriasis rosea and nummular eczema. The pathology report was non diagnostic but suggestive of spongiotic dermatitis. Several weeks later, the rash progressed to grouped follicular keratotic papules with extensive erythema and marked palmarplantar hyperkeratosis. Additional punch biopsies were performed. The pathology showed regular acanthosis and psoriasiform hyperplasia and orthokeratosis consistent with PRP.

The patient then progressed to clinically resemble an acute adult onset PRP with erythroderma, (Figure 1) palmar plantar keratoderma and nails with thick, distal splinter hemorrhages. We considered traditional first-line systemic treatments and begin the patient on 25mg of acitrentin. In an attempt to offer some relief for his generalized erythroderma, the use of topical corticosteroids and emollients were implemented. At the one-month follow-up, the patient’s condition had worsened to the point where he was claiming that “plates of skin” were sloughing off of his body. We added UVB narrowband treatment tri-weekly at Fitzpatrick Skin Type 1 dosing. After no improvement in two months, we added Methotrexate at 15mg for a period of two months. His disease then progressed to include severe palm and sole pain along with extensive palmar plantar hyperkeratosis, with no improvement from the Nb-UVB or MTX. No response was seen after an additional month on methotrexate, which we then opted to discontinue and an oral Phosphodiesterase 4 (PDE4) Inhibitor, apremilast (Otezla; Celgene), was started. The patient remained on apremilast for approximately two months with no marked improvement. Due to the immuno-driven nature of PRP, we considered biologic therapy for the next course of action. After noting the efficacy of secukinumabin psoriasis patients (79% of patients achieved a PASI 90 at week sixteen.) [5], we began the patient on the loading dose of secukinumab: two 150mg syringes injected into the lateral thigh. Four weeks later we were able observe less erythema. He remained on a maintenance dose of 150mg and after eight weeks of continued secukinumab therapy the patient was significantly improved, with only mild erythema but absent of scaling or exfoliation. At his six-month follow-up there was no evidence of disease and the patient had achieved a complete remission.

Citation: Mariasy, Campanelli and Sauder DN. Remission of Pityriasis Rubra Pilaris with IL-17A Antagonist Secukinumab. Austin J Dermatolog. 2016; 3(5): 1063. ISSN:2381-9197