Urticarial vasculitis: A histopathologic and clinical review of 72 cases Darius R. Mehregan, MD, Matthew J. Hall, MBBS,* and Lawrence E. Gibson, MD ~ocheste~ Afinnesota Background: Urticarial vasculitis is a subset of vasculitis characterized clinically by urticarial skin lesions and histologically by necrotizing vasculitis. Objective: A review of patients with urticarial vasculitis was undertaken to further characterize the clinical and histologic findings and to differentiate this disorder from urticaria and other types of cutaneous vasculitis. Methods: Seventy-two cases of biopsy-proven urticarial vasculitis were selected for a review of medical records, laboratory data, and histologic findings. Fifty cases of simple urticaria were also reviewed for purposes of comparison. Results: Systemic symptoms in patients with urticarial vasculitis included angioedema in 30 patients (42%), arthralgias in 35 (49%), pulmonarydiseasein 15 (21 %), and abdominal pain in 12 (17%). Twenty-three patients (32%) had hypocomplementemia. Forty-six ofnpatients (64%) had lesions that lasted more than 24 hours, 23 ofn (32%) had painful or burning lesions, and 25 of 72 (35%) had lesions that resolved with purpura. Sixteen biopsy specimens from the 23 patients with hypocomplementemia showed dermal neutrophilia in addition to the perivascular infiltrate. Of the 23 patients with hypocomplementemia, 20 (87%) had fluorescence of the blood vessels and 16 (70%) had fluorescence of the basement membrane zone as determined by routine direct immunofluorescence. Conclusion: Patients with hypocomplementemia were more likely to have systemic symptoms such as urticaria that resolved with purpura, arthralgias, abdominal pain, and chronic obstructive pulmonary disease. The histologic pattern associated with hypocomplementemia is interstitial neutrophilic infiltrate of the dermis and an immunofluorescent pattern of immunoglobulins or C3 in the blood vessels and along the basement membrane zone. (J AM ACAD DERMATOL 1992;26:441-8.)

Urticarial vasculitis represents a spectrum of disease that is associated clinically with urticaria, but because of vessel damage, it differs histologically from urticaria. As in other cases of cutaneous leukocytoclastic vasculitis, urticarial vasculitis involves the postcapillary venules, and it is considered to be a type III hypersensitivity reaction.' It is~har­ acterized histologically by swelling of endothelial cells, a predominantly polymorphonuclear neutrophilic perivascular infiltrate, leukocytoclasis, extravasation of erythrocytes, and fibrinoid deposits in and around the blood vessels. 2 From the Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester. Accepted for publication Oct. 10, 1991. Reprint requests: Lawrence E. Gibson, MD, Mayo Clinic, 200 First Street SW, Rochester, MN 55905. *Visiting Medical Student Clerk from the University of Sydney, Sydney, AustraJia.

16/1/34305

Clinically, the lesions tend to have a painful or burning sensation, persist more than 24 hours, and may resolve with purpura or hyperpigmentation. 3 Patients may have associated angioedema, arthralgias, abdominal or chest pain, fever, pulmonary disease, renal disease, or episcleritis and uveitis. Urticarial vasculitis has been associated with collagen vascular disease, particularly systemic lupus erythematosus (SLE), complement deficiency, viral infections including hepatitis B, serum sickness, reactions to drugs, and exposure to the sun. 4, 5 Patients with urticarial vasculitis have been categorized into two subgroups: those with hypocomplementemia and those with normal complement levels. Patients with hypocomplementemia are more likely to have systemic involvement than patients with normocomplementemia. 6 Overall, patients with urticarial vasculitis tend to have a benign course. We reviewed 72 cases of urticarial vasculitis evaluated at our institution from 1981 to 1989. Histologic, clinical, laboratory, and direct immunoflu-

441

Journal of the American Academy of Dermatology

442 Mehregan et al.

Table I. Clinical findings at presentation for patients with urticarial vasculitis

~ Pa1ieIItS

Qinical ______ _ _findings _ _ _ _ _ _ _..J

Persistence of urticarial lesions >24 hr