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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 836
Idiopathic hypereosinophilia in an elderly male with contraindications to glucocorticoids and good response to omalizumab


Department of Dermatology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China

Date of Web Publication23-Feb-2023

Correspondence Address:
Ting Zhou
Department of Dermatology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_489_21

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How to cite this article:
Zhou T, Nie X, Pan R, Deng Y. Idiopathic hypereosinophilia in an elderly male with contraindications to glucocorticoids and good response to omalizumab. Indian J Dermatol 2022;67:836

How to cite this URL:
Zhou T, Nie X, Pan R, Deng Y. Idiopathic hypereosinophilia in an elderly male with contraindications to glucocorticoids and good response to omalizumab. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 31];67:836. Available from: https://www.e-ijd.org/text.asp?2022/67/6/836/370303




Sir,

Idiopathic hypereosinophilia is a rare condition characterized by unexplained peripheral blood eosinophilia, often accompanied by elevated immunoglobulin E (IgE) levels. It typically affects middle-aged and elderly populations, in which skin involvement is the most common. Currently, oral glucocorticoids are recommended as first-line therapy. To our best knowledge, we report the first case of idiopathic hypereosinophilia treated by omalizumab, who had contraindications to glucocorticoids.

A 72-year-old male presented at our hospital with concomitant type 2 diabetes mellitus and hypertension with target organ damage. He had a 5-year history of generalized rashes and pruritus severely affected sleep. On examination, blood cell counts were as follows: lymphocyte, 0.79 × 109/L, eosinophil, 1.5 × 109/L, and total immunoglobulin E 457.6 IU/ml. No evidence of infection, malignancy, autoimmune disorders was observed. Other organs showed no abnormalities except aortic and coronary artery calcification. Physical examination revealed scattered, dark red papules and maculopapules on the scalp, limbs, and trunk, some of which were fused into patches [Figure 1]a, [Figure 1]b, [Figure 1]c. Histopathology revealed lymphocytes and eosinophils infiltrating around the superficial dermal blood vessels and appendages [Figure 2]. Bone marrow trephine biopsy showed eosinophilia without any signs of malignancy. FIP1L1-PDGFRA chain fusion and TCR gene rearrangement were not detected. In light of the findings, the patient was diagnosed with idiopathic eosinophilia, and other eosinophilia-associated diseases were excluded. Given that the patient had no complaints of respiratory-related symptoms or neuropathy, eosinophilic granulomatosis with polyangiitis (EGPA) was also ruled out. Oral immunomodulators and antihistamines were almost unhelpful for his symptomatic relief. We decided to initiate treatment with omalizumab at a dose of 300 mg/month subcutaneously as an off-label treatment. Skin lesions almost subsided following 2 months of therapy, leaving pigmentation only [Figure 1]d, [Figure 1]e, [Figure 1]f. The eosinophil returned to normal, but the total IgE was still at a high level (1147 IU/mL), as previous research reported. On remission, the dosing intervals were gradually extended. By months of follow-up, unexpectedly, the efficacy was still maintained without any side effects, and total IgE slowly recovered.
Figure 1: (a-c) Scattered lesions on trunk and limbs. (d-f) Almost complete disappearance of the lesions after 2 months of therapy

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Figure 2: Histopathology showed lymphocytes and eosinophils infiltrating the superficial dermis (a, hematoxylin–eosin stain, ×4; b, hematoxylin–eosin stain, ×20)

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Omalizumab is a humanized monoclonal antibody against IgE, which has been approved for severe allergic asthma and chronic urticaria. Besides, omalizumab has a potential therapeutic effect on other eosinophilic disorders. In a study of omalizumab treatment of eosinophil-associated gastrointestinal disorders, symptom score improved and the eosinophil count decreased in most patients at the middle and end of study.[1] Also, in patients with eosinophilic pneumonia,[2] eosinophilic otitis media,[3] and high IgE syndrome,[4] conditions remission and/or a decrease in absolute eosinophil count were observed after treatment. These may shed light upon the connection between the IgE-mediated process and eosinophilic inflammation. The combination of omalizumab and free IgE leads to downregulation of the expression of high-affinity IgE receptors on the surface of mast cells and basophils,[5] reducing the activation of these cells and the release of mediators including IL-4, IL-3, GM-CSF, IL-5, and IL-13, which serves a central role in promoting the development, survival, trafficking, and activation of eosinophils.

Idiopathic hypereosinophilia tends to occur in middle-aged and elderly populations. In this group, the prevalence of underlying diseases such as diabetes mellitus is higher. Given the side effects of glucocorticoids and some cytotoxic agents, an alternative therapy is required. Considering regression in our case, omalizumab may be considered as a novel treatment option for idiopathic hypereosinophilia.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Foroughi S, Foster B, Kim N, Bernardino LB, Scott LM, Hamilton RG, et al. Anti-IgE treatment of eosinophil-associated gastrointestinal disorders. J Allergy Clin Immunol 2007;120:594-601.  Back to cited text no. 1
    
2.
Kaya H, Gumus S, Ucar E, Aydogan M, Musabak U, Tozkoparan E, et al. Omalizumab as a steroid-sparing agent in chronic eosinophilic pneumonia. Chest 2012;142:513-6.  Back to cited text no. 2
    
3.
Lino Y, Hara M, Hasegawa M, Matsuzawa S, Shinnabe A, Kanazawa H, et al. Clinical efficacy of anti-IgE therapy for eosinophilic otitis media. Otol Neurotol 2012;33:1218-24.  Back to cited text no. 3
    
4.
Chularojanamontri L, Wimoolchart S, Tuchinda P, Kulthanan K, Kiewjoy N. Role of omalizumab in a patient with hyper-IgE syndrome and review dermatologic manifestations. Asian Pac J Allergy Immunol 2009;27:233-6.  Back to cited text no. 4
    
5.
Beck LA, Marcotte GV, MacGlashan D, Togias A, Saini S. Omalizumab-induced reductions in mast cell Fce psilon RI expression and function. J Allergy Clin Immunol 2004;114:527-30.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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