Urticarial vasculitis in the childhood with C2 hypocomplementenemia: a rare case

Area of Science:

• Pediatric Rheumatology
• Immunology
• Dermatology

Background:

• Hypocomplementemic urticarial vasculitis (HUV) is a rare autoimmune disorder.
• It is characterized by recurrent urticarial lesions, vasculitis, and hypocomplementemia.
• Typically, HUV involves low levels of complement proteins, including C1q, C3, and C4.

Observation:

• This report describes a novel pediatric case of HUV.
• The patient presented exclusively with cutaneous manifestations.
• Notably, the hypocomplementemia was specifically linked to the C2 fraction.

Findings:

• This is the first documented instance of HUV with C2 fraction deficiency in a pediatric patient.
• The clinical presentation was limited to skin lesions, distinguishing it from typical HUV cases.
• The specific involvement of the C2 complement fraction offers new insights into HUV pathophysiology.

Implications:

• This case expands the known spectrum of HUV presentations in children.
• Understanding C2 deficiency in HUV may lead to targeted diagnostic approaches.
• Further research is warranted to explore the immunogenetic basis of C2-deficient HUV.