Hypocomplementemic urticarial vasculitis syndrome: a case report and literature review

Abstract

Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case.

Figure 1

Left face and neck. Active raised, erythematous eruption of wheals, particularlyaffecting anterior neck, submandibular area and upper, lower, and cutaneous lips. Note the sparing of the philtrum but not nasolabial fold unlike the malar rash of systemic lupus erythematosus

Figure 2

Right face and neck. Active raised, erythematous eruption of wheals, particularly affecting anterior neck, mandible, and submandibular regions

Figure 3

Anterior wrist and palm. Resolving erythematous wheals soon after flare; urticarial lesions sparing palms with post- inflammatory hyperpigmentation. Note concentration around volar wrist.

Figure 4

Right arm and back (soon after flare). Involvement of upper hips showing both recent resolving erythema. Concentration also at elbows and triceps region

Figure 5

Left calf region. Resolving erythematous wheals soon after flare

Figure 6

Dorsum right hand. Concentration around the metacarpophalangeal joints, less involvement over distal interphalangeal joint and proximal interphalangeal joint

Figure 7

Dorsum hands. Resolving hyperpigmented macules less than one hour after active flare. Concentration around MCP joints, and less so over DIP, PIP, and wrists.