Kimura’s Disease: A Diagnostic Challenge

Abstract

Kimura’s disease is a rare inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E levels. We describe an 11-year-old Asian boy with Kimura’s disease who presented with a chronic left neck mass. The diagnosis was based on the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia and elevated serum immunoglobulin E levels. Pediatricians in western countries should be aware of the clinical presentation of Kimura’s disease.

Kimura Disease: CT and MR Imaging Findings

“…13,15 It has been speculated that a viral or parasitic trigger may alter T-cell immunoregulation or induce an IgE-mediated type 1 hypersensitivity, resulting in the release of eosinophilotrophic cytokines. 13,16 Clinically, KD primarily affects those of Asian descent in their second, third, and fourth decades of life with a male predilection (87%). 6,9,15 Most patients present with firm, painless, single, or multiple subcutaneous lesions in the head and neck, especially in the parotid and submandibular regions.…”

“…9 KD can be confused clinically and histologically with ALHE, also known as epitheloid hemangioma, which is a rare but distinctive vascular tumor typically occurring in women. 16 ALHE, however, usually presents as small erythematous dermal nodules in the head and neck that itch and bleed easily. There is less common association with lymphadenopathy and peripheral eosinophilia.…”

“…Although surgical excision is the preferred method of initial treatment, recurrence is common. 7,12,16,19,20 Other therapeutic options include radiation therapy, systemic corticosteroids, cytotoxic agents, cyclosporin, and pentoxifylline, with variable responses. 16,19,20 The recurrence rate is lower when Ն2 therapies are combined.…”

Cervical lymphadenopathies: a diagnostic crisis

“…8 Renal abnormalities notably nephrotic syndrome and glomerulonephritis are seen in Kimura's disease. 10 Proteinuria is present in 12-16% of cases. 8 CT scan of neck may aid in finding out the extent of the disease.…”

Maladie de kimura: À propos d’un cas

“…Les adénopathies locorégionales non inflammatoires sont quasi-constantes. L'atteinte rénale est présente dans 50% des cas et se manifeste par une protéinurie ou un syndrome néphro-tique lié souvent à une glomérulonéphrite extramembraneuse (6). Par ailleurs, les signes généraux sont absents (4).…”