VisualDx - Diagnosis Details - Fever and Rash: Urticarial Vasculitis

709.1 – Vascular disorders of skin

Urticarial vasculitis is a necrotizing venulitis believed to be caused by deposition of circulating immune complexes, a Type III immune reaction. Fixed urticarial plaques with or without angioedema occur in association with fever, malaise, myalgias, arthralgias, and rarely nausea, vomiting, and diarrhea. The skin lesions are sometimes tender and can itch. Urticarial vasculitis is associated with a variety of systemic disease such as SLE, Sjögren syndrome, serum sickness, and infections (it is rarely reported to be associated with hepatitis B or C). Hypergammaglobulinemia and medications (cimetidine and diltiazem) are cited as a rare cause. The disease is usually self-limited but can persist for years.

Look For

Urticarial, fixed plaques lasting longer than 24–48 hours (idiopathic urticaria always resolves within 48 hours in a specific location). Cayenne-pepper-type pigmentation occurs as sequelae.

Diagnostic Pearls

Unlike urticaria (hives), the lesions of urticarial vasculitis persist in the same location for days to weeks and are sometimes painful.

Differential Diagnosis & Pitfalls

Urticaria
Vasculitis
Capillaritis
Erythema gyratum repens
Subacute cutaneous lupus erythematosus
Erythema marginatum
Erythema annulare centrifigum

Best Tests

Skin biopsy confirms the clinical suspicion.

Management Pearls

Consider a short course of oral corticosteroids.

Therapy

Antihistamines. Plaquenil 200 mg twice daily (obtain baseline visual fields testing and follow up every 6 months if therapy is long-term). Prednisone 20–60 mg per day depending on severity of process.

References

Kaplan AP. Urticara and angiodema. In: Fitzpatrick TB, Wolff K, eds. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill; 2008:330-343.

Brown NA, Carter JD. Urticarial vasculitis. Curr Rheumatol Rep. 2007 Aug;9(4):312-9. PubMed Id: 17688841

Lee JS, Loh TH, Seow SC, Tan SH. Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis. J Am Acad Dermatol. 2007 Jun;56(6):994-1005. PubMed Id: 17504716

Chang S, Carr W. Urticarial vasculitis. Allergy Asthma Proc. 2007 Jan-Feb;28(1):97-100. PubMed Id: 17390766

Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol. 2002 Oct;23(2):201-16. PubMed Id: 12221865

Eads TJ, Fretzin S, Lewis C. Pruritic, painful eruption. Urticarial vasculitis. Arch Dermatol. 1998 Feb;134(2):231, 234. PubMed Id: 9487216

Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992 Mar;26(3 Pt 2):441-8. PubMed Id: 1564151

Associated Findings

Appearance
No Acute Distress
Patient Appears Ill

Body Location
Abdomen
Anterior Neck
Breast
Buttocks
Flank
Forearm
Lateral Neck
Lower Back
Lower Leg
Neck
Sacral Region of Back
Superior Chest
Thigh
Upper Arm
Upper Back

Laboratory
ANA Positive
Erythrocyte Sedimentation Rate (ESR) Elevated
Hypocomplementemia
Leukocytosis
Proteinuria
Rheumatoid Factor Positive

Lesion
Conjunctival Injection (Redness, Bloodshot Eyes)
Petechiae
Wheal (Urticarial Plaque)

Medications
Fluoxetine
NSAID
Potassium Iodide

Signs and Symptoms
Abdominal Pain
Arthralgia (Joint Pain)
Diarrhea
Dyspnea (Shortness of Breath, SOB)
Fever (Febrile)
Headache
Hepatomegaly
Lung Exam - Wheezing
Lymphadenopathy
Malaise
Myalgia (Muscle Pain)
Nausea
Painful Skin Lesions
Splenomegaly
Vomiting

Temporal
Developed Acutely Over Days to Weeks
Developed Chronically Lasting Months to Years
Developed Steadily Over Weeks to Months

Medical History
Hepatitis NOS
Lupus Erythematosus, Systemic
Rheumatoid Arthritis
Sjögren Syndrome