LAUSR

413 (1 mg/kg daily for a week) along with topical corticosteroids with good response. The lip was treated with intralesional corticosteroids (figure 1G). The aetiology of Wells syndrome is unknown. It is thought that an external stimulus increases circulating IL-2, IL-5 and eosinophil cationic protein, activating eosinophils and T helper cells [3]. Various possible triggers of the histological reaction include drugs, arthropod bites, contact dermatitis, and solid and haematological malignancies [4, 5]. Characteristic, but not specific, is the presence of large, severely inflamed, infiltrated plaques associated with a burning sensation and/or pruritus that may be accompanied by fever and myalgia [2]. Different histological features may be observed depending on the timing of the biopsy collection. An eosinophilic infiltrate in the upper and middle dermis without signs of vasculitis can be detected in the acute stage with dermal oedema, while the subacute phase is characterized by ‘flame figures’, eosinophilic major basic protein and fibrin covering collagen fibers [6]. Flame figures are not pathognomonic as they can also occur in other diseases such as eczema, Churg–Strauss syndrome and parasitic infections [7]. In the final stage, eosinophils disappear and histiocytes and giant cells form palisading granulomas [2]. Our patient had an atypical plaque on the lips resembling angioedema, but also typical erythematous non-pruritic plaques on the face and posterior neck, palmoplantar keratoderma with large scaly painful lesions, and increased eosinophils in the peripheral blood but no flame figures on histology. Nevertheless, the marked inflammatory process rich in polymorphonuclear cells, with a predominance of eosinophils and histiocytes, supported the diagnosis. The several treatments used for Wells syndrome exhibit varying degrees of success [3]. Oral steroids are the most effective treatment with prednisone often administered for one week and then tapered over two to three weeks. Topical corticosteroids have also been shown to be effective but should only be considered for limited disease or residual lesions [2]. Antihistamines may be administered to relieve pruritus. To avoid the negative side effects of longterm high-dose steroid use, the anti-inflammatory dapsone has been used alone or as an adjunct to systemic steroids [8]. Despite the generalized involvement, after an initial short course of oral corticosteroids, the patient improved on topical and intralesional corticosteroids. After two years of follow-up, we have not yet identified a cause for the Wells syndrome. The patient remains well controlled on topical corticosteroids, as-needed.