The comprehensive impact of pityriasis rubra pilaris on physical symptoms, mental health, and activities of daily living: A mixed methods study

Abstract

Background Pityriasis rubra pilaris (PRP) is a rare and often severe inflammatory skin disorder with major detriment to patients' quality of life. While past studies have described in detail the morphologic and histopathologic presentations of PRP, little attention has been given to the patient experience. Objectives To identify the physical, mental, and daily living impacts of patients living with PRP Methods Four focus groups involving 47 participants with PRP from six countries were conducted in English. Sessions were recorded and codified by themes, which were used to compose a 23‐question follow‐up survey. Results Common physical symptoms that impacted a majority of PRP patients included severe pruritus, pain (generalised in involved skin, localised to palmoplantar fissures, and arthralgias), poor sleep, fatigue, anhidrosis, body temperature dysregulation, joint pain, buildup of material in the ears, and rhinorrhea. Participants felt that PRP was associated with a strong negative mental impact that caused depression, anxiety, embarrassment, loneliness, loss of self‐esteem, and memory loss or brain fog. 38% endorsed passive suicidal ideation and 4% active suicidal ideation. Common daily activity impacts included loss of fine motor ability and difficulty walking from palmoplantar keratoderma, difficulty wearing normal clothing, difficulty working, and difficulty leaving the home. Participants reported a mean of six applications of topical creams or ointments daily. Conclusions This study illustrates the debilitating daily life impacts of PRP in a variety of domains. In addition to care by a dermatologist, patients should be evaluated for the need for referral to ophthalmology, otolaryngology, and podiatry. Patients with PRP felt strongly that the mental health impact of PRP was not given enough attention by providers. Given the high rate of depression, anxiety, and passive and active suicidal ideation, providers should evaluate patients' mental health and offer a referral to psychiatry and other mental health providers.

Updates on Pityriasis Rubra Pilaris: A Scoping Review

“…A recently published study found that depression is common after diagnosis with PRP, along with a considerable impact on daily living. 5 Additional studies should be conducted to outline the range of impacts PRP has on an individual’s life, allowing clinicians and patients an improved understanding of the expected disease course and support needed.…”

“…Atypical juvenile (Type V) PRP, known as familial PRP, has been potentially linked to Caspase Recruitment Domain Family Member 14 (CARD14) gene mutations. 5 HIV-associated (Type VI) PRP is associated with nodular acneiform lesions, lichen spinulosus-like lesions, and erythroderma. This type is often resistant to therapy and has poor prognosis.…”

Guselkumab for Pityriasis Rubra Pilaris and Dysregulation of IL-23/IL-17 and NFkB Signaling

“…Pityriasis rubra pilaris (PRP) is a rare, often severe, inflammatory papulosquamous disorder characterized by widespread erythematous plaques, furfuraceous scaling, and painful palmoplantar keratoderma with fissuring. PRP has a high degree of morbidity, with a high risk of passive or active suicidality …”

“…PRP has a high degree of morbidity, 1 with a high risk of passive or active suicidality. 2 The pathogenesis of PRP is incompletely understood, but genetic pathways have a strong overlap with psoriasis, 3 including overexpression of interleukin (IL)-23, 4 a proinflammatory cytokine that most prominently activates T helper 17 (Th17) cells. Guselkumab (Tremfya; Janssen Research & Development) binds IL-23p19 and is approved by the US Food and Drug Administration to treat adults with moderate to severe psoriasis.…”