Urticarial Vasculitis

presented by Henry Foong FRCP,

Ipoh, Malaysia

on April 16, 2004

Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia

 
Name
P.W.C., 50-year-old chinese man
Duration
1 month
Distribution
Face,trunk and extremities
History

Presented with recurrent itchy hives on the face, trunk and limbs. Spontaneous onset, the hives were sometimes huge and lasted 2-3 days before subsiding and leaving behind a residual hyperpigmentation. No fever or polyarthralgia. Medication history: nil Past medical history: Mild diabetes on diet and metformin.

Physical Examination

Pertinent findings were annular erythematous wheals 6 x 8cm on the back of neck and trunk. Blanches with pressure. BP 140/80

Images

Laboratory Data

Anti nuclear antibody: negative

Hb 16.2gm% TWBC 11,800 (N55%, L33%, M6%,E0%) Platelets 243 000 ESR 11

Blood glucose 216mg% (12.0mmol/l)

Renal function and urinalysis normal

CXR normal

Histopathology

 

Diagnosis
Urticarial vasculitis
Comments and References
He was started on prednisolone 30mg daily by another dermatologist but the blood sugar level, as expected, increased. Moreover, the patient became uneasy and restless. Fexofenadine 180mg daily together with cetirizine 10mg daily couldn't control the symptoms.

Are you convinced this is urticarial vasculitis? The histology is suggestive but the laboratory tests so far could not pin point the underlying cause. Serology for ANA was negative. There was no relevant drug history.Would adding dapsone 100mg daily be helpful? What is the prognosis for this condition?

Reference:

  1. Davis MDP, Daoud MS, Kirby B, Gibson LE, Rogers RS. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol 1998;38:899-905. Patients with HUV were more likely to be female, to have diffuse neutrophilia on biopsy specimens stained with hematoxylin and eosin, to have continuous strong granular deposition of immunoreactants along the basement membrane zone on DIF, and to have SLE than normocomplementemic patients. We submit that HUV represents a subset of SLE with shared clinical, laboratory, and immunologic features.

Comments from Members

Goh Chee Leok FRCP, Clinical Professor Of Dermatology, National Skin Centre, Singapore

The history here is rather short- 1 month only
The skin lesions are urticarial and suggestive of urticarial vasculitis if it really last for more than 24 hours.

I would wait for another 4 weeks to see if it persist and do further investigations to exclude underlying malignancy or other systemic disease.

I would give him atarax 25-50 mg nocte in addition to the OM doses of non sedative antihistamines. Dapsone is only useful if there is leucocytoclastic infiltrates.

Victoria P Werth, M.D., Associate Professor of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA

It sounds like urticarial vasculitis is a real possibility, and the path seems to support this. You should probably check complements to make sure they aren't low (if low, then this is more consistent with underlying SLE, although this is unlikely given negative ANA). You could treat with either dapsone or colchicine. I usually start with dapsone 50 mg/day and check labs in a week (if G6PD is OK). I then increase by 25 mg/week, rechecking labs to make sure Hb drops aren't too much (about 2 gm drop is OK). Colchicine is another possiblity. I don't think you need prednisone in this situation. Good luck

Jag Bhawan MD, Professor of Dermatology and Pathology, Boston University School of Medicine, Boston, Massachusetts, USA

It is hard to appreciate leucocytoclasia in these images. Some extravasated red cells are seen. However, I think the findings are consistent with urticarial vasculitis. It will be important to make sure that he is not taking over the counter medications and other herbal stuff. Best is to try to find the culprit by process of elimination and history. Dapsone may be a good idea. Since the patient is doing well otherwise, no need to aggressively treat him.You may order RO and LA antibodies to rule out variant of lupus erythematosus.

Yelva Lynfield MD, Cedarhurst, New York, USA on April 17, 2004

Do hepatitis screen and stools for ova and parasites.

Youssef Farid MD, Cairo, Egypt on Apr 25, 2004

The clinical history and the histology fit well with urticarial vasculitis. Ask about drugs like potassium iodide, fluoxetine, and NSAIDs. The oral corticosteroid is of value if there is an associated hypocomplementemic state if not hydroxychloroquine sulfate, dapsone, colchicine or pentoxifylline may be steroid-sparing agents. The antihistaminics are only of value if there is an association of angioedema with the urticarial lesion. So check the complement level first and this will direct you to the correct line of treatment.