Urticaria and Episcleritis in a Woman With Chronic Cough

Abstract

A woman in her 30s presented with a 10-month history of painful pruritic eruption and painful red eyes. The eruption was exacerbated by stress but unrelated to time of day, ambient temperature, or sunlight exposure. Additionally, she reported edema of the dorsal hands and proximal fingers lasting approximately 8 hours during flares without any joint swelling. She also reported recent worsening of a chronic cough. She had an otherwise negative review of systems including denial of any other type of eruption, oral or nasal ulcers, seizures, chest pain, or arthritis. She had no notable medical or family history.A physical examination revealed scattered wheals distributed on her head, trunk, and extremities (Figure , A), with hyperpigmentation and petechiae. Eye examination revealed mild episcerlitis (Figure , B). A punch biopsy was performed (Figure , C and D). Blood work revealed negative results for her antinuclear antibodies (ANA) panel and low levels of C1q, C2, and C3 complement. She also had an elevated C-reactive protein and elevated levels of anti-C1q antibodies (38; normal, <20). Results of urinalysis, complete blood count, and comprehensive metabolic panel were unremarkable; C1-inhibitor levels, anti-double-stranded DNA, hepatitis B virus surface antigen, cryoglobulins, anti-neutrophil cytoplasmic antibodies, and serum protein electrophoresis were within normal limits. Diagnosis B. Hypocomplementemic urticarial vasculitis syndrome Microscopic Findings and Clinical CourseBiopsy revealed fibrin and neutrophils within blood vessel walls surrounded by leukocytoclastic debris, extravasated erythrocytes, and a Scattered hyperpigmented wheals A Original magnification × 20 C Episcleritis B Original magnification × 100 D Figure. A, Physical examination revealed multiple scattered wheals distributed over head, trunks, and extremities. B, Scleral injection of the left eye suggesting episcleritis. C and D, Hematoxylin-eosin. WHAT IS YOUR DIAGNOSIS? A. Systemic lupus erythematosus B. Hypocomplementemic urticarial vasculitis syndrome C. Neutrophilic urticaria D. Familial cold autoinflammatory syndrome Clinical Review & Education