Track

Case Reports

Abstract

Kimura disease (KD) is an uncommon chronic inflammatory disorder of uncertain etiology primarily seen in young or middle-age Asian males that may mimic malignancy.  Its presentation is characterized by painless subcutaneous nodules in the head and neck region that may be accompanied by lymphadenopathy, peripheral blood eosinophilia, and elevated serum IgE levels. Pulmonary involvement has been rarely reported. Herein, we describe a 33-year-old Caucasian male with a progressively enlarging pruritic forehead mass, generalized papular exanthem, lymphadenopathy, peripheral blood eosinophilia, and elevated serum IgE levels. Imaging studies revealed bilateral pulmonary infiltrates with FDG uptake, concerning for chronic eosinophilic pneumonitis. An excisional biopsy of the forehead mass showed histologic findings compatible with KD, including numerous benign-appearing secondary lymphoid follicles with prominent interfollicular eosinophilic infiltrates, and no overtly malignant cells. Immunohistochemistry and flow cytometry did not demonstrate any evidence of a hematolymphoid neoplasm. Genomic testing for myeloproliferative neoplasms, as well as screening for parasitic infections were negative. Treatment with cyclosporine and systemic steroids resulted in the resolution of both the pulmonary infiltrates and cutaneous eruption. This case highlights the importance of considering KD in the differential diagnosis of patients that present with head and neck masses and peripheral blood eosinophilia, and also shows that KD can be accompanied by pulmonary disease. Additionally, we review the available literature regarding the association between KD and systemic and cutaneous manifestations.