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A Review on Pityriasis Rubra Pilaris.
Dingyuan Wang1, Vanessa Cui-Lian Chong2, Wei-Sheng Chong2
1National Skin Centre, 1 Mandalay Rd, Singapore, 308205, Republic of Singapore. dingyuan.wang@mohh.com.sg.
Pityriasis rubra pilaris (PRP) is a rare skin condition. This review covers its subtypes, causes like CARD14 gene mutations, and challenging treatments, highlighting new biologic therapies.
Area of Science:
- Dermatology
- Genetics
- Immunology
Background:
- Pityriasis rubra pilaris (PRP) is an idiopathic papulosquamous dermatosis.
- Characterized by hyperkeratotic follicular papules, scaly plaques, and palmoplantar keratoderma.
- Classified into six subtypes, with subtype VI associated with HIV.
Purpose of the Study:
- To review the clinicopathologic features, pathogenesis, and associated disorders of PRP.
- To critically appraise the existing literature on PRP treatment.
- To emphasize recent advancements in PRP therapy.
Main Methods:
- Literature review of clinicopathologic features, pathogenesis, and treatment of PRP.
- Analysis of Griffiths' classification for PRP subtypes.
- Evaluation of evidence for various therapeutic regimens, including retinoids and biologics.
Main Results:
- PRP presents with distinct clinical features and can be subtyped.
- CARD14 gene mutations are implicated in familial PRP.
- Treatment remains challenging, with oral retinoids as a mainstay and biologics showing promise.
Conclusions:
- Understanding PRP subtypes and associations is crucial for diagnosis and management.
- Further research is needed to establish high-quality evidence for optimal treatment.
- Biologics represent a significant advancement in managing recalcitrant PRP.
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