Diagnostic challenges and updated therapeutic strategies of Kimura’s disease: A case report successfully treated by dupilumab and review

Abstract

Rationale:

Kimura's disease (KD) is a rare and chronic eosinophilic related-disease, characterized by subcutaneous tissue masses, regional enlarged lymph nodes, hypereosinophilia and elevated serum IgE. KD usually affects young adults in the Asian population. In Western countries, the clinical and biological presentation of KD is often unknown, delaying the diagnosis. Therapeutic management is not standardized and despite recent advances, remission from KD can be difficult to achieve, especially in relapse situations.

Patient concerns:

We report the case of an non-Asian man with KD, initially misdiagnosed as lymphoma. We focus on his long-lasting clinical course with 20 years of recurrence despite several therapeutic lines.

Diagnoses and interventions:

We have emphasized the key points of the KD diagnostic challenge. We chose to focus on hemopathies as diagnostic traps to illustrate several overlapping features that blur frontiers with KD. With regard to treatments, lessons can be learned from the use of the therapeutic backbone, which relies on excision surgery, radiotherapy and corticosteroids.

Outcomes:

Advancements in KD pathogenesis have highlighted the pivotal role of Th2 lymphocytes driving eosinophil activation. Directly inspired by eosinophilic and allergic field practices, targeted therapies, such as dupilumab, provide hope for potential curative options.

Lessons:

Finally, we propose a therapeutic plan to treat newly diagnosed KD and discuss options for relapsing entities.