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Relapsing Polychondritis With Palmoplantar Pustulosis
Cloé Comarmond1, Alice Chimon2, Maxime Salfrant3
1Cloé Comarmond, MD, PhD, Department of Internal Medicine and Clinical Immunology, Lariboisière Hospital, Université Paris Cité.
The Journal of Rheumatology
|June 15, 2023
Summary
Palmoplantar pustulosis (PPP) is a rare skin condition linked to relapsing polychondritis (RP). This specific manifestation is not observed in other diseases causing saddle nose, distinguishing it from conditions like granulomatosis with polyangiitis.
Area of Science:
- Dermatology
- Rheumatology
- Immunology
Background:
- Palmoplantar pustulosis (PPP) is a rare skin condition.
- Relapsing polychondritis (RP) is a systemic autoimmune disease affecting cartilage.
- PPP is an uncommon manifestation of RP.
Purpose of the Study:
- To highlight the rarity of Palmoplantar Pustulosis (PPP) in Relapsing Polychondritis (RP).
- To differentiate PPP associated with RP from similar conditions.
- To emphasize the diagnostic significance of PPP in the context of saddle nose deformities.
Main Methods:
- Literature review of rare cutaneous manifestations in autoimmune and autoinflammatory diseases.
- Comparative analysis of clinical features in conditions presenting with saddle nose.
- Case study review focusing on PPP in RP.
Main Results:
- Palmoplantar pustulosis (PPP) is exceptionally rare in relapsing polychondritis (RP).
- PPP is not documented in other conditions causing saddle nose, including granulomatosis with polyangiitis, sarcoidosis, VEXAS syndrome, congenital syphilis, leprosy, or septal abscess.
- The presence of PPP may aid in distinguishing RP from other diseases with similar nasal presentations.
Conclusions:
- Palmoplantar pustulosis is a highly specific cutaneous finding in relapsing polychondritis.
- Differentiating RP from other saddle nose-associated conditions is crucial for accurate diagnosis and management.
- The absence of PPP in other saddle nose conditions underscores its value as a distinguishing clinical marker for RP.
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