• Title/Summary/Keyword: Kimura's disease

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Kimura's Disease of the Distal Arm - A Case Report - (상완 원위부에 발생한 기무라씨 병 - 증례 보고 -)

  • Yoon, Jong-Pil;Jo, Ki-Hyun;Min, Hak-Jin;Yoon, Ui-Seoung;Seo, Jae-Seong;Kim, Jin-Soo;Baak, Cheol-Hee
    • Clinics in Shoulder and Elbow
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    • v.11 no.2
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    • pp.196-200
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    • 2008
  • Kimura's Disease is a very rare, chronic inflammatory disorder with an unknown etiology, and this illness is primarily seen in young Asian males. The typical clinical features consist of painless subcutaneous masses in the head or neck region, and especially in the salivary gland and submandibular region. It is often accompanied with a regional lymphadenopathy, peripheral blood eosinophilia and elevated serum IgE levels. Histologically, it is characterized by prominent germinal centers in the involved lymph nodes, together with eosinophilic infiltration in these lymph nodes. The treatment modalities for this disease are steroid therapy, radiation therapy and surgical excision. Its clinical course is of a benign nature. We report here on a case of a 28 year old man who had a soft tissue mass in his left arm. We excised this mass and the microscopy confirmed that he suffered from Kimura's disease on microscopic examination in his left distal arm.

Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura's Disease

  • Heo, Woon;Jun, Hee Jae;Kang, Do Kyun;Min, Ho-Ki;Hwang, Youn-Ho;Kim, Ji Yong;Nam, Kyung Han
    • Journal of Chest Surgery
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    • v.50 no.2
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    • pp.114-118
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    • 2017
  • Kimura disease (KD) is an immune-mediated chronic inflammatory disease of unknown etiology. KD has many complications associated with hypereosinophilia, including various forms of allergic reactions and eosinophilic lung disease. Additionally, hypereosinophilia is associated with hypercoagulability, which may lead to thromboembolic events. A 36-year-old man with KD presented with acute limb ischemia and coronary artery occlusion. He underwent thrombectomy, partial endarterectomy of both popliteal arteries, and coronary artery stent insertion. KD is a systemic disease that affects many organs and presents with thromboembolism and vasculitis. In a patient with KD, physicians should evaluate the vascular system, including the coronary arteries.

Kimura's Disease of the Elbow - A Case Report - (주관절 부위에 발생한 Kimura씨 병 - 증례 보고 -)

  • Cho, Chul-Hyun;Sohn, Sung-Won;Kang, Chul-Hyung;Oh, Geon-Myung
    • Clinics in Shoulder and Elbow
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    • v.12 no.1
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    • pp.89-93
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    • 2009
  • Purpose: Kimura's disease is an uncommon benign lymphoproliferative inflammatory disorder with an unknown etiology. The recurrence rate after surgical excision is relatively high and renal involvement is its only systemic manifestation. The condition mainly involves the head and neck, and peripheral involvement is extremely rare. Materials and Methods: We encountered the case of a 28-year-old man who had a non-tender mass with mild brownish skin color changes and pruritus around the medial side of the distal arm and elbow. Results: The peripheral blood investigation revealed peripheral eosinophilia and elevated serum IgE levels. Magnetic resonance imaging showed an isointensity signal relative to the muscle on the T1-weighted images and hyperintensity signal relative to the muscle on the T2-weighted images. Conclusion: A marginal resection of the lesion was performed and there was no recurrence at 2 years postoperatively.

ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA : A CASE REPORT (호산구 증가중을 동반한 ANGIOLYMPHOID HYPERPLASIA의 증례보고)

  • Kim, Young-Kyun;Yeo, Hwan-Ho;Lee, Cheol-Woo;Yang, In-Seok;Cho, Se-In;Cho, Jae-O
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.15 no.1
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    • pp.27-34
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    • 1993
  • Angiolymphoid hyperplasia with eosinophilia is an unusual and controversial lesion that occurs primarily in the head and neck area. This lesion was usually confused with Kimura's disease. We present the case of a 32-year-old woman with massive soft movable mass in left facial area which was diagnosed preoperatively as a fasciitis nodular. The final histologic diagnosis of the excised mass was angiolymphoid hyperplasia with eosinophilia(ALHE).

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REPORT OF EXPERIENCE WITH KIMURA'S DISEASE (기무라씨 질환, 5 예 보고)

  • Seel David J.;Park Yoon-Kyu;Lee Kwang-Min
    • Korean Journal of Head & Neck Oncology
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    • v.5 no.1
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    • pp.39-46
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    • 1989
  • Kimura's Disease is a chronic inflammatory and proliferative condition producing subcutaneous masses especially in the head and neck area. This report of our experience with 5 patients with this disease is the first in the Korean surgical literature. Kimura's Disease is thought to be part of the larger spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). It is characterized pathologically by hyperplastic lymphoid follicles, eosinophilic infiltration, and vase 비 ar proliferation. It produces masses which are most common in the area of the parotid, submandibular gland and upper neck. These masses occupy the subcutaneous tissues but also extend into salivary tissue and into upper neck nodes. One of our patients had masses in the groin. The tumors are extremely vascular due to the presence of new proliferative vessels and sinusoids. The average age of our 5 patients was 35, but all but one case were younger than 38 years of age. The male: female ratio was 3 : 2, and the average duration of symptoms was 5,2years. All patients had peripheral blood eosinophilia. All had multiple masses, sometimes symmetrical. The management was surgery alone in one case, surgery and steroids in one case, surgery and radiotherapy in two cases, and all three modalities in one case. The relationship of this entity to ALHE and our experience in the management of this disease are presented. A clinicopathological discrepancy alerted us to the existence of Kimura's Disease. A nineteen-year old male presented with subcutaneous masses over both mastoid areas present for 3 years (Case III). When biopsy on each side was reported as 'eosinophilic granuloma' we submitted the slides to an internationally expert pathologist. Symmetrically occurring tumors in the peri-parotid subcutaneous areas did not fit any category of neoplasm or granuloma known to us. The diagnosis, made by Dr. Gist Fan at the Ochsner Clinic, was Kimura's Disease. We found two additional cases in a review of soft tissue eosinophilic granuloma previously reported at Presbyterian Medical Center, and since then have diagnosed two new cases. These five cases constitute the basis for this, the largest series to be reported in Korea. These vascular, tumor-like lesions of the skin, subcutaneous areas and subjacent structures of the head and neck have been a variety of names, such as angiolymphoid hyperplasia with eosinophilia, eosinophilic hyperplastic lymphogranuloma, angioblastic lymphoid hyperplasia with eosinophilia, histioid hemangioma, and epithelioid hemangioma. The history of this disease spectrum dates back to 1937 when Kimm and Szeto (1) reported 7 cases of 'eosinophilic hyperplastic lymphogranuloma' in the Proceedings of the Chinese Medical Journal. In 1948 Kimura and his associates(2) reported additional cases in Japan under the title 'On the unusual granulation combined with hyperplastic changes of lymphatic tissue.' From then until 1966 several hundred cases were reported in China and Japan. The first report from the West was by Wells and Whimster(3) in the British Journal of Dermatology, in 1969. These authors coined the term, angiolymphoid hyperplasia with eosinophilia (ALHE). Since that time a debate has ensued as to whether Kimura's Disease and ALHE are distinct entities, or whether Kimura's is part of the larger spectrum of ALHE, perhaps a later or advanced phase. From the clinical perspective, surgeons should be aware of the diagnosis of Kimura's Disease not only as part of the differential diagnosis of head and neck tumors but also because these lesions are indolent, and generally require conservative surgical removal as part of the management program. CASE I. A 37-year-old female company employee presented in August 1982 with submental swelling of 12 years' duration and with inguinal swelling of 7 years' duration. The submental mass measured 5x5cm. and the inguinal mass was 8x4cm. in size. Peripheral eosinophilia varying from 14% to 40% was found. On August 20, 1982, the submental mass was removed and a superficial groin dissection was done. In May 1983 an intraoral lesion of the palate was removed. The patient is free of disease. CASE II. A 23-year-old unemployed man visited this hospital for the first time in July, 1984, with swelling of the right cheek present for 6 years. The mass was soft and ill-defined but measured 10x20cm. and extended from the submandibular upper neck to the zygomatic arch, and from the mastoid to the cheek, over the parotid gland. Eosinophilia varying from 27% to 29% was noted in the peripheral blood. On March 21, 1986, the lesion was resected. The procedure comprised an extended superficial parotidectomy from the temporalis fascia to the upper neck. Post-operatively radiotherapy 3000 rad tissue dose was administered using the 6 MeV linear accelerator. The patient remains free of disease. CASE III. A 19-year-old student came to the clinic with masses over both mastoid areas, present 3 years. On the right there were two adjacent lesions, one over the mastoid, the other in the upper jugular level of the neck. On the left it was a single mass over the mastoid. Eosinophilia varied from 13 to 32% in the peripheral blood, and 11.6% in the bone marrow. Incisional biopsy revealed 'eosinophilic granuloma' and a trial of predisolone was employed. The mass increased in size so a small dose of radiation (600 rads) was used, with substantial regression,. The lesion on the left was excised and follwed by 1000 rads radiotherapy. Finally recurrent tumor on the right side was removed on November 5, 1985. The patient remains free of disease. CASE N. A 29-year-old local merchant had had swelling of both upper necks since childhood. At the time of his first visit on March 17, 1986, the right submandibular mass measured 5x3.5cm. and the ,right upper neck and parotid tail mass measured 2.5cm. On the left there were masses in the upper neck, the largest of which measured 2.5cm, and of the parotid tail, 2.0cm. in size.(See Fig. 1) Peripheral eosinophilia of 39% was recorded. Left side partial parotidectomy and resection of the upper neck and subdigstric mases was done on May 2, 1986. The mass involving the right parotid tail and upper neck nodes was removed on Angust 7,1986. Postoperatively the patient was placed on prednisolone 30 mg. per day. No definite masses are palpable. CASE V. A 66-year-old housewife informed us, at the time of her first visit in May, 1986, that she had had multiple neck masses since 10 years ago. On the right side there was a 2.5cm. subcutaneous mass of the upper neck, over the upper jugular chain. On the left there was a 9x4.5cm. mass involving the entire parotid, the post-auricular area and the upper neck. A third mass presented in the submental area and measured 3.5cm. (See Fig. 2) Eosinophilia of 51% was noted in the peripheral blood. partial excision of the left upper neck lesion and complete excision of the submental mass were performed on june 6, 1986. post-operatively she was placed on 20 mg. of prednisolone daily, but when the mass re-grew after two months she was referred to Radiation Therapy for a 2500 rad course of treatment. A barely palpable thickening remains.

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Clinical Study of Choledochal Cyst (총담관낭의 임상적 고찰)

  • Rhim, Si-Yeon;Jung, Pung-Man
    • Advances in pediatric surgery
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    • v.9 no.2
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    • pp.81-88
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    • 2003
  • Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

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Complete Recovery of Oxysterol 7α-Hydroxylase Deficiency by Living Donor Transplantation in a 4-Month-Old Infant: the First Korean Case Report and Literature Review

  • Hong, Jeana;Oh, Seak Hee;Yoo, Han-Wook;Nittono, Hiroshi;Kimura, Akihiko;Kim, Kyung Mo
    • Journal of Korean Medical Science
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    • v.33 no.51
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    • pp.324.1-324.6
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    • 2018
  • Oxysterol $7{\alpha}$-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of $3{\beta}$-monohydroxy-${\Delta}^5$-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol $7{\alpha}$-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.