[Hypocomplementaemic urticarial vasculitis with bullous lesions and pericardial involvement]

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune condition characterized by urticarial vasculitis, low complement levels, and systemic symptoms.
• While systemic manifestations are common, pericardial involvement is exceptionally rare in HUVS.
• This report focuses on a unique case presentation of HUVS with specific pericarditis and bullous lesions.

Observation:

• A 63-year-old woman presented with chronic urticaria, weight loss, respiratory decline, and abdominal pain.
• Skin biopsy revealed leukocytoclastic vasculitis, and laboratory tests showed hypocomplementaemia and anti-C1q antibodies, confirming HUVS diagnosis.
• The patient developed significant pericardial effusion requiring intervention, with pericardial biopsy showing lymphocytic vasculitis.

Findings:

• The case demonstrates an unusual association of HUVS with specific pericarditis and bullous skin lesions.
• Histological examination confirmed lymphocytic vasculitis in the pericardium, a rare finding in HUVS.
• The patient experienced recurrent infectious pneumonitis during follow-up.

Implications:

• This case expands the spectrum of known systemic manifestations of HUVS.
• It underscores the importance of considering cardiac and specific pericardial involvement in HUVS patients.
• Further research may elucidate the mechanisms linking HUVS to specific pericardial and bullous pathologies.