Wells' syndrome: report of a case and review of the literature

Abstract

A 17-year-old woman presented with pruritic papules and plaque wheals on the upper and lower extremities, back, chest, and abdomen, which had appeared 4 months previously. Previous medical history revealed two surgical operations for ovarian cysts (the latter was 2 weeks prior to admittance to the hospital). There was family (brother) but no personal history of atopy, with no known drug allergies. A prior biopsy demonstrated urticaria. Physical examination revealed symmetric, widespread, pink-red plaques with central clearing mostly on the buttocks, but also on the upper and lower extremities and trunk. With pressure, the lesions almost disappeared. Lymph nodes could not be palpated, and all other systems were normal (1-3). Figure 1Open in figure viewerPowerPoint Edematous plaques of Wells' syndrome are seen on the chest and abdomen Figure 2Open in figure viewerPowerPoint Widespread plaques on the back Figure 3Open in figure viewerPowerPoint Close up of exacerbation of lesions on the patient's right arm, breast, and flank Initial laboratory studies revealed the following: hemoglobin 10.8-11.7 g%, white blood cells (WBC) 5210-8070/μL, platelets 402,000/μL, venereal disease research laboratory (VDRL) test negative, cryoglobulins negative, liver and kidney function normal, antinuclear antibodies (ANA) and anti-DNA normal, immunoglobulin E (IgE) 29 IU/mL, complement and other immunoglobulins were within normal values. Urinalysis and urine cultures were normal. There were no parasites in the feces. Sinus and chest X-ray were normal, as was an ear, nose and throat (ENT) examination. An elevated sedimentation rate (23/58) was obtained. A second biopsy revealed features compatible with Wells' syndrome (Fig. 4). Figure 4Open in figure viewerPowerPoint (a) Dense dermal infiltrate composed mainly of eosinophils around blood vessels and between collagen bundles. (b) ‘‘Flame figure’' composed of an aggregate of palisading histiocytes and giant cells surrounding a core of collagen coated with eosinophilic debris Treatment was initiated with prednisone 40 mg for several weeks with tapering of the dose by 5 mg every 3-4 days, along with alumag solution 15 cm3 four times per day.