Urticarial vasculitis: A histopathologic and clinical review of 72 cases

doi:10.1016/0190-9622(92)70069-R

Journal of the American Academy of Dermatology

Volume 26, Issue 3, March 1992, Pages 441-448

https://doi.org/10.1016/0190-9622(92)70069-R Get rights and content

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%), pulmonary disease in 15 (21%), and abdominal pain in 12 (17%). Twenty-three patients (32%) had hypocomplementemia. Forty-six of 72 patients (64%) had lesions that lasted more than 24 hours, 23 of 72 (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 hypocomplementernia, 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 histolog c 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.

References (21)

EW Monroe
Urticarial vasculitis: an updated review
J Am Acad Dermatol
(1981)
NP Sanchez et al.
The clinical and histopathologic spectrums of urticarial vasculitis: study of forty cases
J Am Acad Dermatol
(1982)
WR Gammon
Urticarial vasculitis
Dermatol Clin
(1985)
RK Winkelmann et al.
Diffuse dermal neutrophilia in urticaria
Hum Pathol
(1988)
EW Monroe et al.
Vascultis in chronic urticaria: an immunopathologic study
J Invest Dermatol
(1981)
SF Natbony et al.
Histologic studies of chronic idiopathic urticaria
J Allergy Clin Immunol
(1983)
DK Falk
Pulmonary disease in idiopathic urticarial vasculitis
J Am Acad Dermatol
(1984)
JL Millns et al.
The therapeutic response of urticarial vasculitis to indomethaein
J Am Acad Dermatol
(1980)
LR Lopez et al.
The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine
J Allergy Clin Immunol
(1984)
JS Fortson et al.
Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone
J Am Acad Dermatol
(1986)

There are more references available in the full text version of this article.

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In Urticarial Vasculitis, Long Disease Duration, High Symptom Burden, and High Need for Therapy Are Linked to Low Patient-Reported Quality of Life
2022, Journal of Allergy and Clinical Immunology: In Practice
Urticarial vasculitis (UV) is a rare and difficult-to-treat chronic skin disease defined by long-lasting urticarial lesions and the histopathologic finding of leukocytoclastic vasculitis. As of yet, little is known about UV patients’ perspective on the disease.
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A web-based questionnaire was disseminated in a Facebook group of patients with UV. Patients with UV confirmed by skin biopsy were included.
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Urticarial vasculitis (UV) is a rare cutaneous vasculitis of small vessels characterized by recurrent episodes of wheal-like lesions that tend to last more than 24 hours, healing with a residual ecchymotic postinflammatory hyperpigmentation. The histopathologic pattern of UV is that of leukocytoclastic vasculitis, consisting of fibrinoid necrosis of dermal vessels’ walls and neutrophil-rich perivascular inflammatory infiltrates. Although its etiopahogenesis remains still undefined, UV is now regarded as an immune complex–driven disease with activation of the complement cascade, leading to exaggerated production of anaphylatoxins that are responsible for neutrophil recruitment and activation. This condition can be categorized into 2 main entities according to serum complement levels: normocomplementemic UV and hypocomplementemic UV, the latter being associated with circulating anti-C1q autoantibodies and possible extracutaneous manifestations. Systemic multiorgan involvement may be seen particularly in syndromic hypocomplementemic UV, also known as McDuffie syndrome. This review summarizes the clinicopathological and laboratory features as well as the underlying pathophysiological mechanisms of UV. A focus on its main differential diagnoses is provided, that is, chronic spontaneous urticaria, bullous pemphigoid, IgA (Henoch-Schönlein purpura) and IgM/IgG immune complex vasculitis, lupus erythematous tumidus, Wells syndrome, erythema multiforme, cutaneous mastocytosis, cryopyrin-associated periodic syndromes, and coronavirus disease 2019–associated and anti–severe acute respiratory syndrome coronavirus 2-vaccine–associated urticarial eruptions.
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^*: Visiting Medical Student Clerk from the University of Sydney, Sydney, Australia.

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Urticarial vasculitis: A histopathologic and clinical review of 72 cases

J Am Acad Dermatol

J Am Acad Dermatol

Dermatol Clin

Hum Pathol

J Invest Dermatol

J Allergy Clin Immunol

J Am Acad Dermatol

J Am Acad Dermatol

J Allergy Clin Immunol

J Am Acad Dermatol