A Report on 18 Cases of Children with Kimura Disease in China and Literature Review

Abstract

Background: Kimura’s disease (KD), also known as Eosinophilic Granuloma, is a benign, rare and chronic inflammatory disorder of unknown etiology and it affects subcutaneous tissues, lymph nodes, and salivary glands. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to explore the clinical characteristics, diagnosis and the therapeutic effect of children’s KD in China, in order to improve pediatricians’ knowledge of children’s KD. Methods: The Clinical data of 18 cases of KD diagnosed at Shenzhen Children's Hospital from January 2015 to January 2022 were analyzed retrospectively through case record review. Results: Of 18 cases, 17 were male and one is female. The masses appear as focal, painless, and immovable with an unclear boundary. The most common predilection is head-neck region (n=8, 44.4%). 12 patients showed cutaneous pruritus, 3 patients with nephrotic syndrome and 2 with simple hematuria. 16 patients showed peripheral blood eosinophilia. 12 of 13 patients presented with increased serum Immunoglobulin E (IgE) level. The prominent pathological characteristic is marked lymphoid hyperplasia accompanied by various degrees of vascular hyperplasia and eosinophil infiltration. Among the 18 patients followed for more than 6 months, 9 experienced recurrence of disease after treatment (surgical resection alone: 6/12; oral corticosteroids combined with immunosuppressants: 3/3; surgical resection followed by oral corticosteroids combined with immunosuppressants: 0/3). Through comparison with the clinical features of adult kimura disease, it showed that there was no significant difference between children and adults. Conclusion: KD is characterized by subcutaneous masses but it is also a systemic disease. The diagnosis should be confirmed by pathology. Surgical resection, radiotherapy, hormone or chemotherapy has definite therapeutic effects, but the rate of recurrence in children is high. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

Anti‐immunoglobulin E provides an additional therapy to conventional steroids for Kimura's disease

“…Regarding related mechanisms, we reviewed 13 KD cases that were treated with the abovementioned biologics (Table 1). 5–7,9–15 From these reports, all patients obtained disease remission and none had recurrence during follow‐up. Notably, complications may affect the treatment options and efficacy of KD 7–9,11,14,15 .…”

“…9 However, for those cases without any complications, omalizumab might be suitable. 3,5,12 Nine cases received biologic monotherapies. In particular, Nokana et al 5 reported three patients with KD who responded to omalizumab monotherapy, raising the question of whether biologics should be prescribed alone or in combination with other approaches.…”

“…Case 7 had eczema and responded well to dupilumab, suggesting dupilumab may be an alternative option for KD with allergic conditions 9 . However, for those cases without any complications, omalizumab might be suitable 3,5,12 …”