From the Guidelines
Pityriasis rubra pilaris (PRP) is a rare skin condition characterized by reddish-orange scaly patches, thickened skin, and follicular plugging, with classic features including salmon-colored or orange-red scaly plaques, islands of normal skin, palmoplantar keratoderma, and follicular papules. If you're looking for pictures of this condition, I recommend consulting dermatology resources like DermNet NZ, the American Academy of Dermatology website, or medical textbooks with dermatology sections. These images typically show the distinctive features that differentiate PRP from similar conditions like psoriasis, including the waxy orange-red color, sharp demarcation of lesions, and the characteristic islands of unaffected skin within affected areas 1. Understanding the visual presentation of PRP is important for proper diagnosis, as it can sometimes be confused with other scaling disorders but has distinct treatment approaches including retinoids, methotrexate, or biologics depending on severity and type. Some studies have reported the use of narrowband UVB phototherapy in the treatment of PRP, but its efficacy and safety are not well established, and it should be used with caution 1.
Key Features of Pityriasis Rubra Pilaris
- Reddish-orange scaly patches
- Thickened skin
- Follicular plugging
- Islands of normal skin (islands of sparing)
- Palmoplantar keratoderma (thickening of palms and soles)
- Follicular papules
Diagnosis and Treatment
Diagnosis of PRP is based on clinical presentation and histopathological examination. Treatment approaches vary depending on the severity and type of PRP, and may include retinoids, methotrexate, or biologics 1. It is essential to consult a dermatologist for proper diagnosis and treatment.
Importance of Accurate Diagnosis
Accurate diagnosis of PRP is crucial to differentiate it from other scaling disorders, such as psoriasis, and to initiate appropriate treatment. Misdiagnosis can lead to delayed or inappropriate treatment, which can affect the quality of life and morbidity of patients with PRP. Therefore, it is essential to consult a dermatologist and to use reliable resources, such as DermNet NZ or the American Academy of Dermatology website, to learn more about PRP and its diagnosis and treatment.
From the Research
Characteristics of Pityriasis Rubra
- Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory disease of the skin, which may progress to erythroderma 2
- It is characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules 3
- The disease can present with orange-red confluent plaques, hyperkeratotic follicular papules, palmoplantar keratoderma, and, in some cases, erythroderma 4
- PRP is a chronic papulosquamous disorder of unknown pathogenesis 3
Clinical Presentation
- The diagnosis is based on both clinical and histopathological findings 2
- Clinical presentation can include circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma 3
- Skin biopsy can show acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of PRP 3
Treatment Options
- Treatment options include topical and systemic agents, and physical modalities 2
- Systemic retinoids, particularly acitretin, are commonly used in the treatment of PRP 5, 3
- Biological agents, such as ustekinumab, have also been used in the treatment of PRP 5, 6
- Janus kinase inhibitors, such as upadacitinib, have been reported to be effective in the treatment of PRP 4