Eosinophilic Cellulitis (Wells' Syndrome) Successfully Treated With Low-dose Cyclosporine

Abstract

Eosinophilic cellulitis (EC), or Wells' syndrome, was first described by Wells in 1971 (1). EC is an idiopathic rare disorder representing well-circumscribed erythematous plaques with variable appearances, for instances, papulovesicular, blistering and nodular lesions. There are two stages which comprise acute cellulitic and chronic granulomatous. Limbs are the most prevailing sites and trunk is the next. This disorder tends to be episodic and lasts from weeks to months even to years, and heals without scarring (2-4). Histopathologic picture is characterized by a dense diffuse dermal and sometimes subcutaneous infiltrate predominantly composed of eosinophils. Degranulated eosinophilic materials and nuclear fragments are focally deposited around collagen bundles, forming flame figures, which are distinctive but not solely confined to EC (5, 6). In addition, blood eosinophilia is usually present, albeit variable (2, 4). The patients with EC show variable responses to treatment regimens (2-4). Cyclosporine has been used in the management of some immune-mediated cutaneous diseases including hypereosinophilic dermatoses (7)(8)(9)(10)(11)(12)(13)(14). Thus, we were encouraged to use cyclosporine in attempts to treat recalcitrant EC. Clearance of the lesions resulted from administration of low-dose cyclosporine within 1 month. To our search, this is the first report of patients with EC successfully treated with cyclosporine. We reviewed the pathogenic role of eosinophils and the usefulness of cyclosporine in EC. CASE REPORTS Case 1A 42-yr-old Korean man presented with reddish swollen plaques, measuring 11×17 cm, on the right lower abdomen (Fig. 1). The lesions were slowly increasing in size, and became more edematous and indurated with localized heat sensation. The eruptions had appeared at the same site several times and had persisted for about 1 to 2 months each time. The patient had no specific medical and family history. He denied taking any drug before the lesions had developed. He had neither previous infection symptoms nor insect bites. He had no previous skin disorders other than chronic urticaria. On examination, there was no palpable lymph node at axillary or inguinal areas. Laboratory investigations including ESR, serum glucose, lipid, liver enzymes, BUN, creatinine, uric acid, and electrolytes were within normal limits. White

Successful Treatment of Steroid-Dependent Eosinophilic Cellulitis With Cyclosporine

“…However, the development of side effects led to a switch to cyclosporine. Similarly, two patients refractory to corticosteroids were treated successfully with cyclosporine 10. Cyclosporine acts primarily on helper T cells, decreasing their activation, proliferation, and cytokine production 11.…”

Report of Two Cases with Eosinophilic Cellulitis

“…2,4 Herr ve Koh sis te mik ste ro id, dap son ve mi no sik line ya nıt ver me di ği hal de, oral sik los po rin ile remis yon gö rü len iki ol gu bil dir miş ler dir. 11 Bi zim ol gu la rı mız ise sis te mik ste ro i de iyi ya nıt ver miş -ler dir.…”

Eosinophilic Cellulitis: An 11-Year-Old Male Patient

“…Ancak minosiklinle yan t al nan olgular bildirilmi tir (5). Dü ük doz siklosporinle olumlu yan t al nan olgular (33,34) ve spontan gerileyen olgular da vard r (1,2). Genellikle lezyonlar atrofik plak veya hiperpigmentasyona dönü erek skar b rakmaks z n iyile mektedir ancak literatürde, sekonder enfekte saçl deri yerle imli lezyonlara ba l geli en skarl alopesi bildirilmi tir (7).…”