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Urticarial vasculitis in an infant.
Phoebe E Koch1, Rossitza Lazova, James R Rosen
1Yale University School of Medicine, 333 Cedar St, New Haven, CT 06520-8059, USA.
Cutis
|March 1, 2008
Summary
Urticarial vasculitis (UV), a rare immune complex disorder, typically affects adult women. This case highlights a 9-month-old infant with chronic urticaria diagnosed with UV, emphasizing its rarity in children.
Area of Science:
- Pediatric Dermatology
- Immunology
- Rheumatology
Background:
- Urticarial vasculitis (UV) is a rare immune complex disease, a subtype of leukocytoclastic vasculitis, primarily affecting adult women.
- Characterized by urticarial lesions and decreased serum complement, UV involves small vessel necrotizing vasculitis.
- The condition frequently involves early components of the classical complement cascade, including C1q and C2-C4.
Observation:
- A 9-month-old male infant presented with a 7-month history of chronic urticaria.
- The infant's lesions were initially erythematous wheals, progressing to purpuric papules.
- Lesions resolved with ecchymoses and postinflammatory hyperpigmentation, unresponsive to standard urticaria treatments.
Findings:
- Skin biopsy revealed leukocytoclastic vasculitis, consistent with UV.
- Laboratory studies supported the diagnosis of urticarial vasculitis.
- The clinical presentation and diagnostic findings confirmed UV in this pediatric patient.
Implications:
- This case underscores the importance of considering UV in pediatric patients with chronic, refractory urticaria.
- Highlights the rarity of UV in infants and the need for increased awareness among clinicians.
- Suggests potential complement pathway involvement in pediatric UV cases, warranting further investigation.
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