scholarly journals Kimura’s disease with recurrent bilateral lacrimal gland involvement in a male Japanese child successfully treated with cyclosporine A

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Keisuke Sugimoto ◽  
Takuji Enya ◽  
Yuichi Morimoto ◽  
Rina Oshima ◽  
Kohei Miyazaki ◽  
...  
Keyword(s):  
Cyclosporine A ◽  
Chronic Inflammatory Disease ◽  
Unknown Etiology ◽  
Kimura’S Disease ◽  
Upper Eyelid ◽  
Laboratory Findings ◽  
Eyelid Swelling ◽  
Old Japanese ◽  
Subcutaneous Mass ◽  
The Masses

Abstract Background Kimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, that are typically localized to the neck and head. Involvement of the lacrimal glands and limbs is uncommon and seldom reported. Case presentation We report a case of a 4-year-old Japanese boy presenting with bilateral upper eyelid swelling with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological, and laboratory findings, the patient was diagnosed with KD. An itchy subcutaneous mass on the left arm developed at the age of 14 years. Treatment with steroids was effective. However, as the steroids were tapered after the patient developed side effects, the masses relapsed within a few months. Treatment with cyclosporine A was then initiated, which led to an improvement of clinical features and serial levels of cytokines. Conclusions We report a rare case of KD with a peculiar clinical presentation. The patient responded well to treatment with cyclosporine A.

scholarly journals Kimura’s Disease With Recurrent Bilateral Lacrimal Gland Involvement in a Male Japanese Child Successfully Treated With Cyclosporine A

2021 ◽  
Author(s):  
Keisuke Sugimoto ◽  
Takuji Enya ◽  
Yuichi Morimoto ◽  
Rina Oshima ◽  
Kohei Miyazaki ◽  
...  
Keyword(s):  
Cyclosporine A ◽  
Chronic Inflammatory Disease ◽  
Unknown Etiology ◽  
Kimura’S Disease ◽  
Upper Eyelid ◽  
Laboratory Findings ◽  
Eyelid Swelling ◽  
Old Japanese ◽  
Subcutaneous Mass ◽  
The Masses

Abstract BackgroundKimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, typically localized to the neck and head. Involvement of the lacrimal glands and limbs is uncommon and seldom reported. Case presentationWe report a case of a 4-year-old Japanese boy presenting with bilateral upper eyelid swelling with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological, and laboratory findings, the patient was diagnosed with KD. An itchy subcutaneous mass on the left arm developed at the age of 14 years. Treatment with steroids was effective, but the masses relapsed within a few months as the steroids were tapered after the patient developed side effects. Treatment with cyclosporine A was initiated, which led to the improvement of clinical features and serial levels of cytokines. ConclusionsWe report a rare case of KD with a peculiar clinical presentation. The patient responded well to treatment with CsA.

scholarly journals Unusual presentation of Kimura’s disease: a case series

2017 ◽  
Vol 3 (4) ◽  
pp. 535
Author(s):  
Aarti S. Salunke ◽  
Ravindranath B. Chavan ◽  
Vasudha A. Belgaumkar ◽  
Neelam Bhatt
Keyword(s):  
Case Series ◽  
Neck Region ◽  
Chronic Inflammatory Disease ◽  
Good Prognosis ◽  
Unknown Etiology ◽  
Kimura’S Disease ◽  
Peripheral Eosinophilia ◽  
Unusual Site ◽  
Head And Neck Region ◽  
Subcutaneous Nodules

<p style="margin-bottom: 0in; line-height: 100%;" align="JUSTIFY">Kimura’s disease is a rare chronic inflammatory disease of unknown etiology, presenting as painless subcutaneous nodules with lymphadenopathy and peripheral eosinophilia, mainly disturbing the head and neck region. It mainly affects Asian males in their 2nd to 4th decade of life. Kimura’s disease, although difficult to diagnose clinically, should be considered in the differential diagnosis of patients who have a primary lymphadenopathy with eosinophilia with or without subcutaneous nodules. It should be investigated accordingly as the disease has an indolent course and good prognosis. Herein we report two cases of Kimura’s disease, of which one had unusual site of involvement. </p>

scholarly journals Kimura’s disease mimicking thoracic spine dumbbell neurogenic tumor: a case report and literature review

BMC Surgery ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Siwei Bi ◽  
Jun Gu ◽  
Chenggong Hu
Keyword(s):  
Thoracic Spine ◽  
Neck Region ◽  
Chronic Inflammatory Disease ◽  
Neurogenic Tumor ◽  
Unknown Etiology ◽  
Kimura’S Disease ◽  
Neurogenic Tumors ◽  
Laboratory Examinations ◽  
First Time

Abstract Background Kimura’s disease is a rare, benign chronic inflammatory disease of unknown etiology that mostly affects Asians. The disease typically presents as subcutaneous masses in the head or neck region that are predominantly found in the preauricular and submandibular areas. Case presentation A 7-year-old boy presenting with paralysis of both lower extremities and a thoracic spine dumbbell mass was initially diagnosed with a neurogenic tumor, but the pathological and laboratory examinations confirmed the diagnosis of Kimura’s disease. The paralysis symptom disappeared rapidly, but the patient had developed a recurrent mass in the cervical vertebral canal at the 9-month follow-up. Conclusion To our knowledge, no prior published literature has revealed Kimura’s disease cases that mimic dumbbell neurogenic tumors. Here, we report such a case of Kimura’s disease for the first time and provide a brief review of the literature.

scholarly journals Bilateral Posterior Auricular Masses: A Case of Kimura’s Disease

2021 ◽  
pp. 014556132110455
Author(s):  
Fengyang Xie ◽  
Yan Kou ◽  
ShuaiJun Zhang ◽  
Li Shi ◽  
Jie Han ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Immunoglobulin E ◽  
Elevated Serum ◽  
Asian Population ◽  
Chronic Inflammatory Disease ◽  
Unknown Etiology ◽  
Kimura’S Disease ◽  
Subcutaneous Tissues ◽  
Adults And Children ◽  
Blood Eosinophils

Kimura’s disease (KD) is currently considered a rare chronic inflammatory disease of unknown etiology. It is more common in the Asian population, especially in young and middle-aged men, and can involve the lymph nodes, salivary glands, and subcutaneous tissues. It has been reported in adults and children, and is often accompanied by elevated peripheral blood eosinophils and elevated serum immunoglobulin E (IgE). Herein, we report a case of KD in a 46-year-old man with bilateral masses behind the ears since childhood that had gradually enlarged over 40 years. The patient’s peripheral blood eosinophils were elevated, and interestingly, homocysteine levels were also elevated. After surgical resection of the bilateral posterior auricular masses, follow-up over 5 years indicated good recovery and no signs of recurrence.

scholarly journals Concurrent Pseudomonas Periorbital Necrotizing Fasciitis and Endophthalmitis: A Case Report and Literature Review

Pathogens ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 854
Author(s):  
Yu-Kuei Lee ◽  
Chun-Chieh Lai
Keyword(s):  
Soft Tissue ◽  
Literature Review ◽  
Necrotizing Fasciitis ◽  
Subcutaneous Tissue ◽  
Superficial Fascia ◽  
Upper Eyelid ◽  
Case Presentation ◽  
Phthisis Bulbi ◽  
Eyelid Swelling ◽  
The Rich

(1) Background: Necrotizing fasciitis (NF) is an infection involving the superficial fascia and subcutaneous tissue. Endophthalmitis is an infection within the ocular ball. Herein we report a rare case of concurrent periorbital NF and endophthalmitis, caused by Pseudomonas aeruginosa (PA). We also conducted a literature review related to periorbital PA skin and soft-tissue infections. (2) Case presentation: A 62-year-old male had left upper eyelid swelling and redness; orbital cellulitis was diagnosed. During eyelid debridement, NF with the involvement of the upper Müller’s muscle and levator muscle was noted. The infection soon progressed to scleral ulcers and endophthalmitis. The eye developed phthisis bulbi, despite treatment with intravitreal antibiotics. (3) Conclusions: Immunocompromised individuals are more likely than immunocompetent hosts to be infected by PA. Although periorbital NF is uncommon due to the rich blood supply in the area, the possibility of PA infection should be considered in concurrent periorbital soft-tissue infection and endophthalmitis.

scholarly journals Primary Retroperitoneal Ganglioneuroma: A Retrospective Cohort Study of 32 Patients

2021 ◽  
Vol 8 ◽  
Author(s):  
Jianchun Xiao ◽  
Zixuan Zhao ◽  
Binglu Li ◽  
Taiping Zhang
Keyword(s):  
Growth Pattern ◽  
Clinical Manifestations ◽  
Incomplete Resection ◽  
Radiological Imaging ◽  
College Hospital ◽  
Laboratory Findings ◽  
Female Ratio ◽  
Medical College ◽  
The Mean ◽  
The Masses

Purpose: To investigate the clinical characteristics, diagnosis, differential diagnosis, therapy options, and outcomes of retroperitoneal ganglioneuroma.Methods: In this retrospective study, we collected and analyzed the clinical data of 32 patients diagnosed with retroperitoneal ganglioneuroma and admitted to Peking Union Medical College Hospital from October 2012 to August 2019.Results: Among our 32 cases with retroperitoneal ganglioneuroma, the male-to-female ratio was 1:3 and the mean age was 35. Only 25% of the cases presented with abdominal pain while more than 65% had no specific symptoms. The masses could be found through physical examination in only five patients. Most of the tumors are located near the renal area. They were usually single and displayed an embedded growth pattern with diameters &lt;10 cm, clear borders, and soft texture. For radiological imaging, the majority of tumors demonstrated soft tissue density with mild-to-moderate enhancement on CT imaging and showed hypoecho with moderate blood flow signals in ultrasound. No significantly abnormal laboratory examinations were found in most patients. Of all the 32 patients, 2 chose surveillance after biopsy due to difficulties in operation, while others chose surgical resection. The mean follow-up time was 15.8 months among 26 patients. The tumor remained stable in the surveillance cases. Residual tumors were found in four cases receiving operations with no progress and discomfort. No recurrence was seen in all patients.Conclusions: The retroperitoneal ganglioneuroma is a benign tumor without specific clinical manifestations or significant laboratory findings. Typically, it is shown as low density with a clear border and an embedded growth pattern in radiological imaging. The overall prognosis is good. Surgery is an effective approach with possible severe complications. Incomplete resection or surveillance can be considered for some cases where complete resection is difficult to achieve.

scholarly journals Lichen Planus as a Possible Rare Cause of an Ocular Disease

2019 ◽  
Vol 75 (4) ◽  
pp. 219-223
Author(s):  
Jana Nekolová ◽  
Markéta Středová ◽  
Miroslav Podhola ◽  
Naďa Jirásková
Keyword(s):  
Lichen Planus ◽  
Inflammatory Disease ◽  
Ocular Surface ◽  
Negative Impact ◽  
Rare Event ◽  
Chronic Inflammatory Disease ◽  
Mental Condition ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Irreversible Damage

Lichen planus is a relatively common mucocutaneous papulosquamous disorder of unknown etiology that is thought to be immunologically mediated. Ocular involvement of lichen planus (LP) is a rare event, which can lead to irreversible damage to the ocular surface and loss of vision from cicatrizing blepharoconjunctivitis and corneal scarring. Steroids are the first treatment choice. Treatment with topical ciclosporin A is also a possible treatment. We report a case of 79-year-old woman who was treated for long lasting severe inflammatory disease of a ocular surface. It led to blindness of one eye and low vision of the second eye. The diagnosis was made thanks to correlation of biopsy results from oral and ocular surface. The patient was also diagnosed with middle-stage dementia. Negative impact of steroid therapy on mental condition of this patient was considered. On the other hand, dementia could cause worse compliance with recommended treatment regime. Chronic inflammatory disease of ocular surface requires long lasting treatment very often and it is sometimes not as effective as expected. Discovering etiological causes of the disease is a priority for the right treatment. We emphasize also the key role of physical and mental condition in management of ocular surface disease.

Treating Severe Pediatric Keratoconjunctivitis with Topical Cyclosporine A

2021 ◽  
Author(s):  
Julia Biermann ◽  
Friederike Bosche ◽  
Nicole Eter ◽  
Flemming Beisse
Keyword(s):  
Cyclosporine A ◽  
Treatment Plan ◽  
Corneal Opacity ◽  
Corneal Neovascularization ◽  
Visual Development ◽  
Vernal Keratoconjunctivitis ◽  
Upper Eyelid ◽  
Sparing Effect ◽  
One Year

AbstractThe incidence of chronic keratoconjunctivitis, which potentially causes long-term loss of visual acuity due to corneal opacity, is considerably less common in children than in adults. It is therefore in danger of being overlooked. In children the appropriate treatment is therefore often introduced too late, or to an insufficient extent. In this article we would like to raise awareness about the diagnosis of chronic keratoconjunctivitis in children, and to present an effective treatment plan for severe stages of the disease. There are two forms of chronic keratoconjunctivitis that occur most frequently in children: hyperergic blepharokeratoconjunctivitis (hBKC) and vernal keratoconjunctivitis (VKC). With hBKC, the patient often has a history of recurring hordeolum and also presents with blepharitis; it is characterized by the marked presence of corneal neovascularization in the lower circumference of the cornea. VKC is typically characterized by changes under the upper eyelid, with marked changes to the superior limbus. If there is a risk of complications involving the cornea, or in the presence of such complications, a consistent long-term topical immunosuppressive and anti-inflammatory treatment is required. Both of these properties are combined in the active ingredient cyclosporine A. Other advantages of topical CSA treatment are its steroid-sparing effect and the long-term reduction of exacerbations. Parents need to be informed about the chronic nature of these two diseases and their tendency to recur; because of these characteristics, treatment, in most cases, should be envisaged for at least one year in order to effectively disrupt the complex immunologic processes. This safeguards the childʼs visual development and prevents amblyopia caused by scarring and astigmatism. We hope that the data presented will lower the barriers related to prescribing CSA for topical eye application in children.

scholarly journals Two Dog-Related Infections Leading to Death: Overwhelming Capnocytophaga canimorsus Sepsis in a Patient With Cystic Echinococcosis

Medicina ◽  
2012 ◽  
Vol 48 (2) ◽  
pp. 11 ◽  
Author(s):  
Raimonda Matulionytė ◽  
Ingrida Lisauskienė ◽  
Gintautas Kėkštas ◽  
Arvydas Ambrozaitis
Keyword(s):  
Echinococcus Granulosus ◽  
Cystic Echinococcosis ◽  
Unknown Etiology ◽  
Healthy Individuals ◽  
Capnocytophaga Canimorsus ◽  
Laboratory Findings ◽  
Oral Flora ◽  
Recent Exposure ◽  
The Family ◽  
Fatal Sepsis

Capnocytophaga canimorsus is a fastidious, capnophilic, fusiform, and filamentous gram-negative rod. It is part of the normal oral flora of dogs and cats and can cause an infection in humans, but is of generally low virulence in healthy individuals. A case of fatal sepsis due to Capnocytophaga canimorsus in a 46-year-old woman with clinically silent cystic echinococcosis discovered postmortem is present. She had been bitten by a dog 3 days before the symptoms appeared. The family had owned the dog for 4 years. A preliminary diagnosis of septic shock of unknown etiology with multisystem organ failure was established. Despite all the efforts, the patient died on the seventh day of hospitalization. Laboratory findings received postmortem showed Capnocytophaga canimorsus isolated from the blood culture after 7 incubation days. Autopsy showed a cyst in the liver with a fibrotic wall and necrotic eosinophilic interiors containing fragments of Echinococcus granulosus scolices. In conclusion, an interaction possibly established long ago between the host and Echinococcus granulosus conditioned immunosuppression mechanisms developed by the parasite in this case, which can explain such an aggressive course of the infection with Capnocytophaga. Two dog-related infections were fatal in the middle-aged dog owner considered healthy before this hospitalization. Vigilance concerning recent exposure to dogs or cats and potential immunosuppression risk factors must be maintained in a patient presenting with clinical features of fulminant sepsis.

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