Steroid Dependent Recalcitrant Well's Syndrome in a Patient with Chronic Lymphocytic Leukemia (CLL) Responding to Hydroxychloroquine (HCQS)

Abstract

IntroductionWell's syndrome is an uncommon skin condition with cosmetically unpleasant outcomes. It was initially described by Dr. GC wells in 1971 as recurrent granulomatous dermatitis with eosinophilia(1).CaseA 69-years-old woman with well controlled CLL presented with pruritic, papular eruptions & ulcerations over extremities and back (Figure 1). She denied asthma or atopy, insect bite, or new medications. Skin biopsy (Figure 2) revealed perivascular & interstitial infiltrate composed of lymphocytes, histiocytes, neutrophils and eosinophils.Antibiotics, antihistamines, dapsone, steroid injection failed to control her symptoms. She responded to oral prednisone. On attempted steroid taper she experienced disease flares. She was transitioned eventually to HCQS. Prednisone was discontinued. She remained in remission on HCQS.DiscussionWell's syndrome is often described as exaggerated response to insect bite; it may occur without an inciting event, commonly involves extremities, may precede or follow the diagnosis of CLL and has no association with disease course (2). The lesions range from itchy papules, plaques to edematous vesiculo-bullous lesions and panniculitis or ulcerations (3) and can result in disfiguring skin atrophy (4). Pathology reveals eosinophilic or lymphohistiocytic infiltrates (5). Use of glucocorticoids, antihistamines, immunoglobulins, dapsone, ultra violet-B therapy for treatment has been described (2, 5) and may require prolonged treatment (6). Multiple recurrences are often seen in recalcitrant cases (7).Well's syndrome should be considered in differential diagnosis of skin lesions in patients with CLL. HCQS offers an alternative to long term steroid use in its management.