A case of Vogt-Koyanagi-Harada syndrome

( Han Mi Jung ),( Hyun Jee Kim ),( Joo Hee Lee ),( Ji Hae Lee ),( Jung Min Bae ),( Gyong Moon Kim ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic disease that affects tissues containing melanin. The disease is characterized by chronic bilateral panuveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The classic clinical course of VKHS can be divided into 3 stages: Stage 1 includes headache, fever, and photophobia; Stage 2 includes bilateral uveitis, hypoacusis, meningitis, and cutaneous involvement with poliosis, vitiligo, and alopecia; and Stage 3 can last for weeks or months or be chronic. Early and aggressive treatment with systemic corticosteroids is the main stay of the initial therapy. A 39-year-old healthy female presented with headache, ocular pain and visual defect for 6 weeks. Ophthalmologic examinations revealed retinitis with pigmented epithelial detachment of retina. And high white blood cell count in cerebrospinal fluid was observed. She was diagnosed as Vogt-Koyanagi-Harada syndrome. The neurologic and opthalmic symptoms were relieved with steroid pulse therapy (prednisolone 1000 mg/day for 5 days) but after tapering the steroid pulse therapy, several skin lesions with hypopigmented patches on the forehead and arms showed up. For vitiligo, the patient is being treated with methylprednisolone 16 mg/day, topical tacrolimus 0.1%, narrow-band Ultraviolet B phototherapy. Herein, we report a case of typical features of VKH syndrome.

A case of Vogt-Koyanagi-Harada (VKH) syndrome with alopecia universalis in an adolescent girl

( Ji Yoon Choo ),( Young Hoon Yoon ),( Ji Hyun Lee ),( Young Min Park ),( Jun Young Lee ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Vogt-Koyanagi-Harada (VKH) syndrome is a rare systemic disorder and an idiopathic autoimmune disease characterized by panuveitis in association with cutaneous, neurologic, and auditory abnormalities. It usually affects darker-pigmented individuals, especially members of the Asian, Indian, and Latin races. Women are more affected than men (2:1), and the peak incidence is seen in the third or fourth decade of life. It is rarely seen in children, with one study indicating that only 3% of all VKH cases are observed in childhood. The cutaneous features are alopecia and vitiligo or poliosis, dependent upon the site of melanocyte location. Alopecia usually appears as discrete, alopecic patches around the head. However, in our knowledge, only a few cases have been reported diffuse hair loss and only one previous case has been reported alopecia universalis with VKH syndrome. A 13-year-old woman presented with asymptomatic, diffuse hair loss and depigmented patches on the scalp since 1 year ago. She had been suffered total hair loss on the whole body after 2 months. She had uveitis, meningitis and tinnitus for several years, but no family history. According to clinical manifestation, diagnosis of VKH disease was established. Herein, we report a rare case of VKH syndrome with alopecia universalis in an adolescent period.

스테로이드 감량 치료 중 재발을 보인 보크트-고야나기-하라다증후군의 임상양상

김지수,김동윤,김경태,채주병 대한안과학회 2019 대한안과학회지 Vol.60 No.4

Purpose: To evaluate the clinical features of recurrent Vogt-Koyanagi-Harada syndrome during steroid tapering after high-dose intravenous steroid therapy. Methods: We retrospectively reviewed the medical records of eight patients who were diagnosed and treated for Vogt-Koyanagi- Harada syndrome from January 2010 to December 2017. Recurrence was defined as the occurrence of uveitis or serous retinal detachment during oral steroid tapering. We grouped the patients into two groups according to recurrence, and compared the best-corrected visual acuity, maximum height of subretinal fluid, central subfield thickness, choroidal thickness, average steroid tapering period and images from optical coherence tomography. Results: Seventeen patients were included and five patients (29.4%) recurred during oral steroid tapering. Patients with recurrent episodes were treated with increasing doses of oral steroid and/or oral immunosuppressant and sub-tenon injection of triamcinolone. Serous retinal detachment was completely absorbed after 24 weeks of treatment in all 17 patients, including the recurrence group and the best-corrected visual acuity was not significantly different between the two groups. The heights of the subretinal fluid and thicknesses of the central subfield before treatment were significantly higher in the recurrence group than in the non-recurrence group. Conclusions: Serous retinal detachment can recur during oral steroid tapering after high-dose intravenous steroid treatment in patients with Vogt-Koyanagi-Harada syndrome suggesting that the height of the subretinal fluid at the fovea and central subfield thickness before treatment are factors associated with recurrence. Even in the case of recurrence, good visual prognosis can be expected after additional treatment according to the clinical situation. 목적: 고용량 정맥 스테로이드 치료 후 경구 스테로이드로 감량 중 재발하는 보크트-고야나기-하라다증후군의 임상양상을 알아보고자 한다. 대상과 방법: 2010년 1월부터 2017년 12월까지 보크트-고야나기-하라다증후군으로 진단받아 치료받은 환자 17명, 33안을 대상으로 연속적 후향적 의무기록을 분석하였다. 스테로이드 감량 치료 중 포도막염이나 장액망막박리가 다시 발생한 경우를 재발이라 정의하고, 재발한 군과 비재발군의 치료 전, 치료 후 24주째의 최대교정시력과 최대 망막하액의 높이, 중심망막두께, 맥락막두께, 스테로이드 감량 치료 기간, 빛간섭단층촬영 등을 분석하였다. 결과: 5명(8안)에서 경구 스테로이드 감량 치료 중 사라졌던 망막하액이 다시 관찰되었으며 재발한 환자군은 경구 스테로이드 증량, 경구 면역억제제 복용, 테논낭하 트리암시놀론 주사를 상황에 맞게 병용하였다. 사라졌던 망막하액이 다시 관찰된 환자를 포함하여, 17명의 환자 모두 치료 24주 후 장액망막박리가 완전히 흡수되었으며, 최대교정시력은 두 군에서 유의한 차이를 보이지 않았다. 치료 전 최대 망막하액의 높이와 중심망막두께는 재발군에서 비재발군에 비해 통계적으로 유의하게 높게 측정되었다(p=0.04). 결론: 보크트-고야나기-하라다증후군에서 고용량 정맥 스테로이드 치료 후 경구 스테로이드 감량 시 사라졌던 장액망막박리가 재발할 수 있으며, 망막중심오목에서 치료 전 최대 망막하액의 높이와 중심망막두께는 재발과 관련있는 인자로 추측된다. 재발한 경우에도 상황에 맞는 적극적인 치료를 통해 좋은 시력예후를 기대할 수 있을 것으로 생각한다.

VKH 증후군과 유사한 양안 다발성 장액성 망막박리를 보인 양측성 부신 림프종

김인태,박혜영,노영정,In Tae Kim,Hae Young Park,Young Jung Roh 대한안과학회 2008 대한안과학회지 Vol.49 No.7

Purpose: To report a case of bilateral adrenal gland lymphoma mimicking symptoms of Vogt-Koyanagi- Harada syndrome. Case summary: A 57-year-old woman complained of decreased visual acuity and metamorphopsia in both eyes. She had no prior medical history, but had experienced headaches in the past week. Fundus examination revealed multiple serous retinal detachment. Fluorescein angiography of the fundus revealed multiple pinpoint leaks in early stages and subretinal dye pooling in the retinal detachment area in late stages. The most likely diagnosis was therefore Vogt-Koyanagi-Harada syndrome. Two days later, mild fever developed and a systemic evaluation was performed. Computed tomography of chest and abdomen showed bilateral adrenal gland T-cell lymphoma. Through prompt combination chemotherapy, the patient’s systemic condition recovered, and her fundus recovered with improved visual acuity. Conclusions: We experienced a rare case of bilateral adrenal lymphoma which masqueraded as Vogt-Koyanagi- Harada syndrome. When there are systemic symptoms such as mild fever and headaches, systemic evaluation should be done so as not to miss more serious systemic pathology. J Korean Ophthalmol Soc 49(7):1198-1202, 2008

건선과 동반된 보그트-고야나기-하라다 증후군

윤정현 ( Jeong Hyun Yun ),이지연 ( Ji Yeoun Lee ),윤태영 ( Tae Young Yoon ) 대한피부과학회 2008 大韓皮膚科學會誌 Vol.46 No.1

어지럼증과 청력저하를 동반한 하라다병 1예

한정욱,노웅재,박태정,김보영 대한이비인후과학회 2011 대한이비인후과학회지 두경부외과학 Vol.54 No.3

Vogt-Koyanagi-Harada (VKH) syndrome is an uncommon multisystem autoimmune disease affecting the melanocytes located in the uvea, inner ear, skin, hair, and meninges. The syndrome is frequently reported by ophthalmologists since the most consistent finding is uveitis. Hearing loss, tinnitus and vertigo are the symptoms of VKH syndrome. The authors report one case of VKH syndrome with hearing loss and vertigo with related literature.

보그트-고야나기-하라다병 환자에서 한약 단독치료로 시신경유두 부종이 감소한 치험 1례

이동진,권강,서형식,Lee, Dong-Jin,Kwon, Kang,Seo, Hyung-Sik 대한한방안이비인후피부과학회 2014 한방안이비인후피부과학회지 Vol.27 No.2

Objectives : Vogt-Koyanagi-Harada(VKH) syndrome is a multisystem autoimmune disease characterized by its affects on pigmented tissues in the ocular, auditory, integumentary and central nervous system. This case is to report a decreased optical disc swelling in VKH patient treated only with herbal medication. Methods : A 56-year-old male patient with VKH experienced headache and numbness during 7 months of steroid treatment. He visited our clinic and was administered Haeulsoyo-san (解鬱逍遙散) three times a day for a month. No other oriental or western treatments were included. Result & Conclusions : Patient didn't complain any discomfort during the treatment. Significantly decreased optical disc swelling was confirmed by taking fundus photo. This is the first case of KVH to be reported in Korean medicine society.

전신탈모증을 보인 Vogt-Koyanagi-Harada 증후군

구자웅 ( Ja Woong Goo ),이석준 ( Seok Joon Lee ),최성원 ( Seung Won Choi ),이원수 ( Won Soo Lee ) 대한피부과학회 2006 大韓皮膚科學會誌 Vol.44 No.10

박복되는 현훈으로 발현한 보크트-고야나기-하라다 증후군 1예

이태경,남상원,박선아,성기범 대한평형의학회 2003 Research in Vestibular Science Vol.2 No.2

재발성 보크트-고야나기-하라다 증후군에 대한 임상 분석

김민정,조남천,안민,Min Jung Kim,Nam Chun Cho,Min Ahn 대한안과학회 2006 대한안과학회지 Vol.47 No.2

Purpose: We analyzed the clinical features of patients with recurrent Vogt-Koyanagi-Harada(VKH) syndrome. Methods: A retrospective chart review of 21 patients diagnosed with VKH syndrome between 1994 and 2004 was performed. We grouped the patients into two groups according to the duration of ocular inflammation. Group A included patients with recurrent ocular inflammation lasting 24 months or longer and Group B included those with a duration of less than 24 months. We compared the clinical manifestations, responses to treatment and complications. Results: Group A had 7 patients and Group B had 14 patients. There were significant differences in initial visual acuity, the interval between ocular symptoms and treatment, total steroid dose, and complications. The average final visual acuity of Group A was 0.572 (0.2-1.0), which was relatively favorable. Conclusions: The initial visual acuity and the interval between ocular symptoms and treatment seemed to be important predictors. We conclude that aggressive treatment is necessary in recurrent VKH syndrome, as visual prognosis was relatively favorable despite the cost of the problems associated with high dose steroids and ocular complications.