Wells syndrome associated with chronic lymphocytic leukemia

Abstract

Eosinophilic cellulitis or Wells syndrome is an uncommon skin condition of unknown etiology that can occur alone or associated with other conditions. Typically, it presents with recurrent pruritic, erythematous and edematous plaques, but it can also show clinical polymorphism. Besides the cutaneous lesions, patients can experience systemic manifestations like fever, malaise, arthralgia and peripheral blood eosinophilia. We describe a case of this rare syndrome that presented with polymorphic cutaneous lesions associated with a serious systemic disease, which was revealed through the investigation of the cutaneous disease.

Canine acute eosinophilic dermatitis with oedema (Wells‐like syndrome) associated with T‐cell multicentric lymphoma

“…Although it was impossible to confirm a causal relation in a definitive manner in the present case, the following reasons make it likely that the relapsing multicentric lymphoma acted as a trigger for the onset of CAEDO: (1) correlation between the onset of the dermatologic signs and relapse of the lymphoma; (2) significant cutaneous improvement with the chemotherapy treatment that was contemporaneous with the reinduction of remission from the lymphoma and (3) suspected link between EC (which is a similar condition to CAEDO) and lymphoma in human literature. Notably, as with several reports in people,22 24 26 28 29 31 33 the patient’s skin lesions only seemed to improve following treatment of the malignancy, with complete clinical cure achieved after six weeks on lomustine chemotherapy. Although confirming it is impossible, the authors speculate whether this observation could help explaining the lack of response to glucocorticoid therapy noticed early in this case, as trigger‐focused therapy would be needed to observe clinical improvement.…”

“…However, in human medicine, there are multiple reports associating EC with non‐haematological and haematological malignancies (table 1). In the majority of these cases (10/15), clinical signs of EC preceded the initial diagnosis of the neoplastic process,22–31 diagnosis of metastasis32 or diagnosis of relapse of malignancy 30…”

“…Non‐Hodgkin lymphomas Chronic lymphocytic leukaemia27 28 33 39 40 Mantle‐cell lymphoma29 30 Angioimmunoblastic lymphadenopathy31 …”

Case of Wells’ syndrome: A rare association with the clinical course of chronic lymphocytic leukemia

“…5 However, one case, who had developed Wells' syndrome before the diagnosis of CLL, had similar cutaneous manifestations to that of our case. 3 In our case, Wells' syndrome had developed in the clinical course of CLL, and diffuse erythematous plaques had been relieved by the treatment for CLL without the additional administration of prednisolone. We speculate that an elevation of serum IL-5 in CLL may activate eosinophils and results in the formation of those cutaneous manifestations.…”

“…1,2 The development of Wells' syndrome in the clinical course of chronic lymphocytic leukemia (CLL) is rare. 3 Here, we present a rare case of Wells' syndrome with blisters associated with CLL. A 58-year-old man presented with swelling and bullae on his upper limbs.…”