Treatment Options for Juvenile Pityriasis Rubra Pilaris

Successful treatment of CARD14‐negative juvenile Pityriasis rubra pilaris with Ustekinumab

“…While ustekinumab has been described in several cases of CARD14-mutated juvenile PRP and is discussed as potential novel first-line therapy, its role in CARD14-wildtype patients remains unclear. 4 Here, we demonstrated a successful treatment initiation with a lasting effect 1 year after its start. Further case reports or, if possible, controlled randomized trials are needed to confirm our observations, enhance therapy management and to gain a better understanding of the pathogenesis of autoinflammatory keratinization in juvenile PRP.…”

“…4 CARD14 variants are seen as a predisposing factor to autoinflammatory keratinization, as seen in juvenile PRP. [4][5][6] Here, we report the successful treatment with ustekinumab in a juvenile PRP patient without CARD14 mutation, proposing to overthink the potential range of indication.…”

Case report: Area of focus of management of severe pityriasis rubra pilaris by dose optimization of adalimumab biosimilar in combination with acitretin and montelukast

“…Due to its rarity, the potential for spontaneous remission and the absence of specific scoring tools, there is a dearth of clinical trials and comparative studies addressing treatment options ( 2 ). At the same time, numerous case reports and case series have highlighted the positive impact of TNFα inhibitors, as well as other biologics such as IL-12/23 inhibitors, IL-17 inhibitors, and IL-23 inhibitors, in both adults and children with PRP, either as standalone or combined therapies ( 1–3 , 9–13 ), Pathogenically, the IL-23/IL-7 axis appears to play a significant role ( 1 , 14 ). Gain-of-function mutations in the CARD14 gene, notably associated with familial PRP in humans, have led to heightened IL-17 and IL-23 inflammation in mice ( 1 , 14 ).…”

“…At the same time, numerous case reports and case series have highlighted the positive impact of TNFα inhibitors, as well as other biologics such as IL-12/23 inhibitors, IL-17 inhibitors, and IL-23 inhibitors, in both adults and children with PRP, either as standalone or combined therapies ( 1–3 , 9–13 ), Pathogenically, the IL-23/IL-7 axis appears to play a significant role ( 1 , 14 ). Gain-of-function mutations in the CARD14 gene, notably associated with familial PRP in humans, have led to heightened IL-17 and IL-23 inflammation in mice ( 1 , 14 ). Reviews that compare the effectiveness of systemic therapies, particularly biologics alone or in conjunction with conventional treatments, have demonstrated complete or excellent responses as follows ( 2 , 3 ): infliximab 80 and 57.1%, etanercept 81.8 and 53.3%, adalimumab 77.8 and 46.4%, and ustekinumab 92.3 and 62.5% ( 2 , 3 ).…”

“…While spontaneous resolution within three years is possible, treating persistent cases can be challenging ( 2 ). Systemic retinoids are recommended as first-line therapy, with conventional immunosuppressants being the second-line option in treatment-resistant cases ( 1 , 3 ). Biologics are often proposed as a third-line treatment.…”