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Pityriasis rubra pilaris as a systemic disease
Jivko Kamarachev1, Ivan Grozdev2, Razvigor Darlenski3
1Department of Dermatology, Venereology and Allergology, University of Zurich, Zurich, Switzerland.
Clinics in Dermatology
|December 23, 2019
Summary
Pityriasis rubra pilaris (PRP) is a rare inflammatory skin condition. Research suggests PRP may be linked to systemic diseases through common immune pathways.
Area of Science:
- Dermatology
- Immunology
- Rheumatology
Background:
- Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder.
- Characterized by follicular papules, scaly plaques, and palmoplantar keratoderma.
- Etiology remains largely unknown.
Purpose of the Study:
- To explore the potential systemic nature of Pityriasis rubra pilaris.
- To investigate associations between skin and extracutaneous manifestations.
- To examine underlying pathomechanisms linking skin and systemic involvement.
Main Methods:
- Review of clinical heterogeneity in PRP.
- Analysis of reported rheumatologic, infectious, and neoplastic associations.
- Hypothesis formulation regarding common pathomechanisms.
Main Results:
- PRP primarily affects the skin but shows clinical heterogeneity.
- Associations with various extracutaneous conditions are noted.
- Evidence supports links to systemic immunologic processes and superantigen mimicry.
Conclusions:
- PRP may extend beyond a skin-limited disorder.
- A causative relationship between skin and systemic manifestations is hypothesized.
- Common immunologic pathways may underlie PRP's diverse manifestations.
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