Kimura Disease of the Thigh Treated With Surgical Excision and Dupilumab

Huang, Hsuan-Yu; Yang, Chin‐Yi; Yao, Wen‐Teng; Chen, Yu-Fan; Yu, Chia‐Meng; Tung, Kwang‐Yi; Tsai, Ming‐Feng

doi:10.1097/sap.0000000000003106

Cited by 17 publications

(22 citation statements)

References 17 publications

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“…Further reports were found for Kimura’s disease with 4 patients treated with dupilumab and 3/4 experiencing complete and one partial remission [ 232 , 233 , 234 , 235 ]. Four cases of steroid-refractory papuloerythroderma Ofuji treated with dupilumab all reported complete resolution of skin lesions and pruritus [ 236 , 237 , 238 ].…”

Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the signaling of interleukin (IL)-4 and -13. Several other chronic skin conditions share mechanistic overlaps with AD in their pathophysiology, i.e., are linked to type 2 inflammation. Most recently, dupilumab was approved by the U.S. Food and Drug Administration for prurigo nodularis (PN). Given its relatively good safety profile, effective off-label use of dupilumab has been reported for a multitude of dermatologic diseases and several clinical trials for dermatologic skin conditions are currently ongoing. We conducted a systematic review of applications of dupilumab in dermatology other than AD and PN by searching the databases PubMed/Medline, Scopus, Web of Science and Cochrane Library as well as the clinical trial registry ClinicalTrials.gov. We found several reports for effective treatment of bullous autoimmune diseases, eczema, prurigo, alopecia areata, chronic spontaneous urticaria, Netherton syndrome and a variety of other chronic inflammatory skin diseases.

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Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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“…The most frequently affected areas are the head and neck region, especially the parotid glands, salivary glands, and lymph nodes (9). Masses in other areas, such as the groin (10), breast (11), arm (12), thigh (13), and orbit (14), have also been reported. This study was consistent with previous studies that most lesions (14/16) were located in the head and neck region, but there were still two cases located in the back and elbow, respectively.…”

Section: Discussionmentioning

confidence: 99%

“…In this study, six patients received combination therapy, and no recurrence has been reported until now, which is in line with the previous results. Several biological agents, such as benralizumab (41), an anti-IL-5 receptor antibody; mepolizumab, an anti-IL5 antibody (42); and dupilumab, a Th2 pathway-targeted agent (13), have been successfully used in the treatment of KD on a small scale, but the efficacy in pediatric patients still needs further validation.…”

Section: Discussionmentioning

confidence: 99%

Kimura disease in children: A report of 11 cases and review of the literature

Mai

Wang²,

Sun³

et al. 2023

Front. Pediatr.

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IntroductionKimura disease (KD), also known as eosinophilic hyperplastic lymphoid granuloma, is a rare benign chronic inflammatory condition, which is featured with the painless progressive mass located in the subcutaneous area of the head and neck region, elevated peripheral blood eosinophils, and raised serum immunoglobulin E (IgE) levels. KD is uncommon in clinical practice, especially with low incidence in children, so it often leads to misdiagnosis or missed diagnosis in pediatric patients.MethodsThe clinical data of 11 pediatric patients with KD in the authors' institution were retrospectively analyzed.ResultsThere were 11 pediatric patients with KD enrolled in total, including 9 male patients and 2 female patients (sex ratio 4.5:1). The median age at diagnosis stood at 14 years (range 5-18 years), the initial symptoms in all patients included painless subcutaneous masses and focal swelling, the duration of symptoms ranged from 1 month to 10 years, and the average duration was 24.45 months. Six patients had single lesions, and 5 had multiple lesions. The highest proportion of lesion regions were parotid gland (n = 5, 31.3%) and retroauricular (n = 5, 31.3%), followed by cervical lymph nodes (n = 4, 25%), and others (n = 2,12.5; elbow n = 1; back n = 1). The eosinophil absolute count elevated in all patients, ranging from 0.71×109/L to 10.35 ×109/L (normal range 0.02-0.52×109/L). IgE levels were increased in all 7 patients who underwent serum immunoglobulin examination (normal range <100 IU/mL). Three patients received oral corticosteroid treatment while 2 relapsed. Three patients received surgical resection combined with oral corticosteroid treatment, and no patient relapsed. The other 3 patients received surgery and radiotherapy, surgery combined with corticosteroid and cyclosporin and corticosteroid combined with leflunomide respectively, and no patient relapsed.ConclusionBase on the study, it is found that Kimura disease is rare and may have the atypical symptoms in pediatric patients, combination therapy is recommended to reduce recurrence, and long-term follow-up should be performed.

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“…Recently, Szeto et al demonstrated a successful treatment of benralizumab with the long-standing efficacy of clinical improvement (19). Dupilumab also has been applied by Hsuan-Yu Huang et al and Yuichi Teraki et al with complete relief after 8-10 month of therapy (20,21).…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review

Yang

Yu²,

Jia³

et al. 2022

Front. Immunol.

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Kimura disease (KD) is a rare and benign chronic inflammatory disease of unknown cause. It is characterized by subcutaneous granuloma of soft tissues in the head and neck region, increased eosinophil count, and elevated serum IgE. Currently, no definitive treatments are recommended. A 57-year-old Chinese man was diagnosed with KD after 7 years of slow subcutaneous masses growth. The patient underwent treatment of oral glucocorticoids for 1 year, but the masses recurred as the dosage was tapered down. Subsequent anti-IgE therapy of omalizumab administered subcutaneously at 450 mg/day at a 4-week interval did not show improvement. The size of masses and serum IgE and circulating eosinophils did not decrease significantly after 19 cycles of continuous treatment. Ultimately, switched strategy of dupilumab was applied at an initial dose of 600 mg, followed by 300 mg every 2 weeks for 4 months. This treatment demonstrated dramatical effects with reduced masses in each area and fast dropdown of eosinophil counts, while the high level of serum IgE remained without changes. Recently, different biologics including anti-IgE, anti-IL-5, and anti-IL-4/IL-13 have been applied to treat KD with satisfied results and help to explore the pathogenesis of this rare disease. To our knowledge, this is the first report that demonstrates the effects of two different biologics in the same patient and reveals the impressive clinical efficacy of dupilumab to treat KD independent of IgE. Therefore, further investigation of the underlying mechanism and the development of diagnosis and treatment of KD is valuable.

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Kimura Disease of the Thigh Treated With Surgical Excision and Dupilumab

Cited by 17 publications

References 17 publications

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Kimura disease in children: A report of 11 cases and review of the literature

Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review

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