Kimura disease with Allergic Bronchopulmonary Aspergillosis: a case report

KD was first described as "eosinophilic hyperplastic lymphogranuloma” by Kimm and Szeto (China) in 1937, but it was first reported definitively by Kimura (Japan) in 1948 and has been widely known as Kimura disease since then [6]. It is endemic to Asia and tends to affect young men aged 20–40 years. But it has no strict restrictions on ethnicity, age, and gender. The previous reports have showed that sporadic cases were also found in Spain, Saudi Arabia, South America, and other non-Asians. The youngest patient was a 15-month old African American boy [7]. Although it is recognized that two typical laboratory examinations are elevated peripheral eosinophil and serum IgE, Vivek et al. reported a case of KD without peripheral eosinophilia [4]. KD is characterized by subcutaneous masses in the head and neck region, sometimes with local lymph node enlargement and salivary gland involvement. Rare cases can involve the orbit and ocular appendages, epiglottis, armpit, long bone, breast, groin, genitals, mediastinum. In this case, the patient’s age was not consistent with the classic age of onset reported in previous literature. Our patient presented rare multiple enlarged inguinal lymph nodes. KD is a systemic disease that can usually involve kidneys and skin, which are usually manifested as nephrotic syndrome, skin pruritus, or rashes, respectively. Our patient had normal liver and kidney function, but there were obvious itching and rashes on the trunk and extremities. It had been reported that eosinophils contain various amounts of tissue factor (TF) and some researchers had concluded that relatively high TF in patients with hypereosinophilia might lead to an increased thrombotic risk [8]. A patient with KD was reported to be complicated with acute limb ischemia and coronary artery disease [9]. The D-dimer in the patient we reported was at a high level. Vascular US examination of both lower extremities showed bilateral femoral atherosclerotic plaques formation, suggesting he had a high risk of thrombosis. Therefore, with the consent of the patient, we prescribed a 0.4 ml heparin sodium injection for prophylactic anticoagulation. The coagulation function result revealed D-dimer was 1.5 mg/L before he was discharged from the hospital.

KD is a benign disease of unknown etiology and pathogenesis. However, the increased peripheral eosinophil and serum IgE have oriented that KD might be associated with autoimmunity, insect bites or infections, and allergies caused by parasites [10, 11]. Otah N et al. have revealed that Th2 and Tc1 cells instead of Th1 and Tc2 cells contribute to the pathogenesis of KD [12]. Katagiri K et al. have found that the expression levels of IL-4, IL-5, IL-13, and IFN-γ mRNAs in peripheral blood mononuclear cells in a patient with KD are significantly higher than after surgery or radiotherapy, which supports Th2 cytokines play a role in the development of KD [13]. In addition, Agarwal R et al. have reviewed that the immune response in ABPA is a Th2 CD4+ T cell response, accompanied by increased secretion of cytokines such as IL-4, IL-5, and IL-13. The Th2 immune response leads to the synthesis eosinophils and IgE (including total and A. fumigatus specific) [5]. So we suspected that there was a certain correlation between the pathogenesis of these two diseases rather than an incidental. To our knowledge, this is the first case of KD combined with ABPA. Based on the order of onset of the two diseases, we have reasonable speculation that ABPA may induce the occurrence of KD, but the speculation needs more clinical evidence to confirm. When reviewing the patient's history, we unexpectedly found the patient’s chest CT examination showed several enlarged lymph nodes in the left axilla as early December 2018 (Fig. 5a). He didn’t have any relative discomfort at that time, it subsided without symptomatic treatment in August 2020 (Fig. 5b). This time the right inguinal lymph nodes enlargement occurred, which manifested KD was self-limited and recurrent with an indolent course and good prognosis [14].

Fig. 5figure 5

Chest CT scan: a Several enlarged lymph nodes could be seen in the left armpit in December 2018 (red arrow). b No enlarged lymph nodes found in the left armpit in August 2020

The definite diagnosis of KD depends on histopathologic examination. Our initial diagnosis suspicion was lymphoma, but the histopathologic results helped us get an accurate diagnosis. The imaging findings, including CT, magnetic resonance imaging (MRI), and US are non-specific, but they are helpful to deter-mine the lesion morphology, anatomical distribution. Yang et al. indicated that the ill-defined, infiltrative lesions on imaging were associated with a higher recurrence rate [15]. Differential diagnosis of KD includes angiolymphoid hyperplasia with eosinophilia (ALHE), cutaneous IgG4-related disease (IgG4-RD), Hodgkin's lymphoma, Langerhans cell histiocytosis, florid follicular hyperplasia, Castleman's disease, dermatopathic lymphadenopathy [16]. The main differential diagnosis is ALHE which shares similar histological features with KD, however, patients with ALHE typically have normal eosinophils and IgE level [17].

There is no standard treatment protocol for KD to date. Treatment includes surgical resection, regional or systemic steroids, antihistamines, immunosuppressant including cyclosporine (CsA) or anti-IgE antibody (omalizumab), or radiotherapy [18, 19]. The individualized treatment plan should be selected according to the clinical manifestation of patients. For patients with KD combined with renal involvement, steroids may be the best treatment [20]. Surgical resection is preferred for primary lesions without multiple system involvement [21].

Kimura disease is a chronic disease with a prolonged course and a high recurrence rate. No malignant transformation has been reported till date. KD involves multiple disciplines such as immunology, dermatology, nephrology, and otolaryngology. Its different clinical manifestations have varying degrees of impact on patient’s lives, including pruritus, renal function damage, disfigurement, vascular embolism. Rare cases and atypical manifestations of KD make the early diagnosis difficult. Our unique case may give some clinicians the inspiration to explore the relationship between KD and ABPA. Multidisciplinary team should pay more attention to collect clinical data to study the etiology of KD.

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