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

Omigawa, Chika; Namiki, Takeshi; Ueno, Makiko; Ugajin, Tsukasa; Miura, Keiko; Yokozeki, Hiroo

doi:10.1111/1346-8138.14579

Cited by 5 publications

(5 citation statements)

References 4 publications

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“…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.…”

Section: Discussionsupporting

confidence: 80%

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

Alves¹,

Foale²,

Cerundolo³

et al. 2020

Vet Record Case Reports

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A nine-year-old female neutered Boxer in clinical remission from T-cell multicentric lymphoma was presented for further investigation of multiple, non-painful, non-pruritic, oedematous, erythematous, serpiginous plaques. On examination, a very mild right superficial cervical lymphadenomegaly was also detected. Initial glucocorticoid treatment for a suspected hypersensitivity reaction was unsuccessful and the patient presented a week later with worsening of the dermatological signs. Cytology of peripheral lymph nodes was consistent with lymphoma relapse. Histopathology of skin biopsies was compatible with canine acute eosinophilic dermatitis with oedema, also known as Wells-like syndrome. Complete clinical resolution of the skin lesions and the peripheral lymphadenomegaly was achieved with six weeks of lomustine chemotherapy. To the authors’ knowledge, this is the first description of canine acute eosinophilic dermatitis with oedema suspected to be triggered by a multicentric lymphoma in a dog.

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Section: Discussionsupporting

confidence: 80%

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

Section: Discussionmentioning

confidence: 99%

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

Alves¹,

Foale²,

Cerundolo³

et al. 2020

Vet Record Case Reports

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“…Wells as "recurrent granulomatous dermatitis with eosinophilia" [55], WS is a rare dermatosis without ethnic or gender predilection that primarily affects adults [56]. The etiology is unknown, although some cases have been associated with various underlying conditions and triggers, including hematologic disorders [20,[57][58][59][60][61][62][63][64][65], hypersensitivity reaction to insect bites [40,66,67], medications [68][69][70], infections [71][72][73], and vaccinations [56,[74][75][76]. Peripheral eosinophilia, leukocytosis, or elevated inflammatory markers may be observed [58,77].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, bites or stings from arthropods such as mosquitoes, fleas, honeybees, ticks, spiders, and even centipedes have been reported as potential triggers of WS [38,40,59,66,67,83]. Similarly, hematologic malignancies have been mentioned as potential initiators of WS [20,[56][57][58][59][60][61][62][63][64][65]84].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Dermatosis of Hematologic Malignancy: Emerging Evidence for the Role of Insect Bites—A Retrospective Clinico-Pathological Study of 35 Cases

Michelerio,

Rubatto,

Roccuzzo

et al. 2024

JCM

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Background/Objectives: Eosinophilic dermatosis of hematologic malignancy (EDHM) is a rare cutaneous disorder associated with various hematologic malignancies, most commonly chronic lymphocytic leukemia. Detailed clinicopathologic studies of EDHM are lacking and the pathogenesis remains enigmatic. Initially thought to be a hypersensitivity reaction to insect stings, subsequent reports have challenged this understanding. The prognostic implications of EDHM remain unclear. Methods: A retrospective clinicopathologic study was performed on patients diagnosed with EDHM. Hematologic and dermatologic data were reviewed. Histologic specimens were re-evaluated and lesions were classified into acute/subacute, fully developed, and chronic/regressing. Results: The study included 35 patients. In 80% of these patients, EDHM was diagnosed after the hematologic disorder. Approximately 45% of the cohort experienced hematologic disease progression or relapse, while 65% required therapeutic intervention during the course of their hematologic disease. In total, 15/19 CLL patients had non-mutated IgHV, a marker of a more aggressive hematologic disease course. Dermatologic lesion morphology was heterogeneous, with most lesions occurring on exposed areas, and a significant 94% of patients demonstrated lesion seasonality. Histopathologic findings were consistent with features typically associated with insect bites. In addition, examination of lesions at different chronological stages revealed substantial similarities with Wells syndrome. Conclusions: Our findings support the potential role of insect bites in triggering EDHM in the context of adaptive immune dysfunction. EDHM may be associated with a more aggressive disease course or may be a marker of disease progression. The observed co-occurrence of features typical of Wells syndrome in EDHM patients suggests that these conditions are part of a spectrum of disorders that vary in clinical expression.

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“…These include arthropod bites and stings, medications, metals, as well as bacterial, viral, fungal, parasitic, and helminthic infections [ 3 – 5 ]. It has been associated with leukemia, lymphoma, myeloproliferative syndromes, solid tumors, Churg-Strauss syndrome, and can overlap hypereosinophilic syndrome [ 6 – 8 ]. A possible type IV hypersensitivity reaction in response to a variety of exogenous and endogenous stimuli has also been suggested [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Recurrent Cellulitis-Like Episodes of the Lower Limbs and Acute Diarrhea in a 30-Year-Old Woman: A Case Report

et al. 2021

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Patient: Female, 30-year-old Final Diagnosis: Wells syndrome Symptoms: Acute diarrhea • chronic recurrent episodes resembling cellulitis to her lower limbs bs Medication: — Clinical Procedure: Skin biopsy Specialty: Dermatology • Family Medicine Objective: Unusual clinical course Background: Wells syndrome is an uncommon inflammatory dermatosis that presents as tender or mildly pruritic cellulitis-like eruptions. The clinical presentation can include papular and nodular eruptions, annular plaques, vesicles, bullae, and urticaria. This syndrome can be promoted by inappropriate eosinophilic stimulation by factors that affect normal eosinophil cellular systems. Histologically, during the acute phase, a dense infiltrate of degranulating eosinophils is located in the epidermis and dermis. Wells syndrome mostly has a benign course; however, its recurrences can be frequent over the years. It can be an imitator of bacterial cellulitis leading to ineffective antibiotic courses, which can promote a Clostridioides difficile infection. Case Report: A 30-year-old woman presented with chronic episodes resembling cellulitis on her lower limbs, for the past 5 years. Her past medical and family history was insignificant. She had been prescribed several courses of antibiotics. At this presentation, she had edema in the lower part of her left foot, erythema and pruritus, mild diarrhea, and fever for 4 days. She was diagnosed with a Clostridioides difficile infection with a background of Wells syndrome. She was successfully treated for Clostridioides difficile with metronidazole and probiotics, and had complete clinical remission of the Wells syndrome with mometasone furoate cream and levocetirizine. Conclusions: This report emphasizes the need for greater vigilance by physicians before making a final diagnosis of infectious cellulitis. Thus, excluding from their differential diagnosis, other dermatological diseases mimicking infectious cellulitis and preventing a possible misdiagnosis, delay of appropriate treatment, future antibiotic-associated complications, and the evolution of antimicrobial-resistant microbes in the community.

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

Cited by 5 publications

References 4 publications

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

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

Eosinophilic Dermatosis of Hematologic Malignancy: Emerging Evidence for the Role of Insect Bites—A Retrospective Clinico-Pathological Study of 35 Cases

Recurrent Cellulitis-Like Episodes of the Lower Limbs and Acute Diarrhea in a 30-Year-Old Woman: A Case Report

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