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

References:

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)

References:

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.

CHRONIC LOCALIZED FIBROSING LEUKOCYTOCLASTIC VASCULITIS Specific Disorders:

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

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