Urticarial Vasculitis

Synonyms: Urticarial venulitis; hypocomplementemic or normocomplementemic urticarial vasculitis; hypocomplementemic urticarial vasculitis syndrome.

Clinical Presentation (Fig. 8):

■ Women more than men (3:2-4:1), fourth decade peak incidence

■ <20% of patients presenting with chronic urticaria have urticarial vasculitis (UV)

■ Cutaneous differences between UV and urticaria

■ Painful, tender, burning or pruritic papules, and plaques

■ Urticaria is mostly pruritic

■ Lesions persist less than 24 hours and greater than 72 hours

■ Urticaria lasts between 8 to 24 hours

■ Residual purpura or hyperpigmentation

■ No residua in urticaria

■ No site predilection

■ Urticaria more common on lower extremities

■ Systemic signs include: low-grade fever, angioedema, arthralgias and arthritis, and abdominal pain

■ Hypocomplementemia can be observed in 18% to 32%

■ Hypocomplementemic UV have more severe disease compared to normocomplementemic UV:

■ Mostly female

■ Tissue neutrophilia and +lupus band test on skin biopsy

■ Coexisting connective tissue disease, frequently systemic lupus erythematosus

■ hypocomplementemic urticarial vasculitis syndrome:

■ Arthralgias or arthritis

■ Glomerulonephritis

■ Uveitis or episcleritis

■ Recurrent abdominal pain

■ Obstructive lung disease

■ Up to 70% of UV patients with + lupus band test have renal disease (e.g., membranoproliferative glomerulonephritis, focal necrotizing vasculitis)

■ Sparse perivascular and interstitial neutrophilic infiltrate with either:

■ Focal small vessel neutrophilic vasculitis, or

■ Focal perivascular neutrophilic nuclear debris without fibrin deposits, and with or without extrava-sated red blood cells.

■ Minimal histologic criteria for the diagnosis of UV:

■ Nuclear debris or fibrin deposits, with or without extravasated red blood cells

■ Diffuse neutrophilic infiltrates more common in hypo-complementemic UV

■ Dermal eosinophils more common in normocomple-mentemic UV

■ DIF evaluation reveals vascular C3 and/or immunoglobulins, mostly IgM, in 47%

■ Vascular immunoreactants found frequently in patients with hypocomplementemic UV, 87% to 100%

■ Normocomplementemic UV have significantly less frequent vascular immunoreactants, 29%

■ Positive lupus band test (basement membrane zone deposits of C3 and/or immunoglobulin ranges) ranges from 18% to 34% in all UV patients

■ Hypcomplementemic UV, 70% to 96% + lupus band test

■ Normocomplementemic UV, 1% to 18% + lupus band test


1. Davis MD, Daoud MS, Kirby B, Gibson LE, Rogers RS III. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol 1998; 38(6 Pt 1):899-905.

2. Davis MD, Brewer JD. Urticarial vasculitis and hypocomple-mentemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 2004; 24(2):183-213.

3. Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histo-pathologic and clinical review of 72 cases. J Am Acad Dermatol 1992; 26(3 Pt 2):441-448.

4. Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore) 1995; 74(1):24-41.

SEPTIC VASCULITIS Clinical Presentation (Fig. 9):

■ Acute vasculitis caused by septic states due to infective endocarditis or infection with gonococci, meningococci, pseudomonads, staphylococci, streptococci, certain rick-ettsial infections, and other microorganism

■ Clinical lesions are characterized by:

■ Purpura (petechiae and ecchymoses)

■ Vesiculopustules, often with grey roofs signifying necrosis

■ Hemorrhagic bullae

■ Ulceration, rarely

■ Chronic gonococcemia and chronic meningococcemia patients have triad of

■ Intermittent fewer

■ Arthralgias

■ Fewer clinical lesions, distributed over extremities, particularly acral surfaces, which are mostly:

■ Petechiae surrounded by a rim of erythema

■ Vesiculopustules with grey necrotic roof

■ Hemorrhagic bullae, rarely

■ Mixed neutrophilic small and muscular vessel vasculitis with deep dermal and subcutaneous vessel involvement

■ Scant perivascular fibrin

■ Muscular arteritis common (Fig. 9D, block arrow)

■ No or little nuclear debris (differentiates septic vasculitis)

■ Epidermal spongiosis, intraepidermal vesicles, and pustules common

■ Neutrophils within or underlying the epidermis

■ Eosinophils absent or sparse

■ Organisms are rare in skin lesions except in acute meningococcemia

■ Vascular immune complexes can be detected by direct immunofluorescence

■ Necrosis of adnexal sturctures (Fig. 9C)


1. Chan HL. Bacterial infections of the skin. II: cutaneous clues to systemic infections. Ann Acad Med Singapore 1983; 12(1): 98-102.

2. Chiller K, Selkin BA, Murakawa GJ. Skin microflora and bacterial infections of the skin. J Investig Dermatol Symp Proc 2001; 6(3):170-174.

3. Plaut ME. Staphylococcal septicemia and pustular purpura. Report of cases. Arch Dermatol 1969; 99(1):82-85.

4. Rodriguez-Pla A, Stone JH. Vasculitis and systemic infections. Curr Opin Rheumatol 2006; 18(1):39-47.

5. Somer T, Finegold SM. Vasculitides associated with infections, immunization, and antimicrobial drugs. Clin Infect Dis 1995; 20(4):1010-1036.


Granuloma faciale, erythema elevatum diutinum, and some cutaneous inflammatory pseudotumors.

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

Get My Free Ebook

Post a comment