Multiple xanthogranuloma in an adult patient

( Hye Sung Han ),( Ga Ram Ahn ),( Kui Young Park ),( Seong Jun Seo ),( Myeung Nam Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Xanthogranuloma is a benign, asymptomatic and self-healing disorder of non-Langerhans cell histiocytosis clinically characterized by multiple yellowish papules. This condition predominantly occurs in infancy and childhood, as it is also known as juvenile xanthogranuloma. Only around 10% of xanthogranuloma cases manifest in adulthood. Unlike juvenile xanthogranuloma, adult xanthogranuloma usually presents with solitary cutaneous lesion which does not resolve spontaneously. A 34-year-old man presented with multiple dusky red to brownish colored, dome shaped nodules on the right inguinal area which persisted for 8 years. Through the histopathological findings which revealed infiltration of multinucleated giant cells including foamy Touton cells, xanthogranuloma was diagnosed. Herein, we report an unusual case of adult-onset multiple xanthogranuloma.

Eruptive xanthogranuloma in a healthy adult male

( Dongyoung Roh ),( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Gun-wook Kim ),( Hoon-soo Kim ),( Byung-soo Kim ),( Moon-bum Kim ),( Hyun-chang Ko ),( Won-ku Lee ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although rarely occurs in adults. Lesions in the adult form are typically solitary. Multifocal eruptive presentation of xanthogranuloma in adult is very rare. We report a rare case of eruptive xanthogranuloma in a healthy man. A 31-year-old male presented with multiple erythematous to yellowish papules on trunk for 2 months. The lesions were asymptomatic, but the number of the lesion gradually increased. The patient had no other general symptom. On physical examination, 20 to 30 discrete, reddish to yellow, dome-shaped and flat-topped, 0.5 to 1 cm papules and nodules were observed on the chest, abdomen and extremities. Serum lipid studies were normal. Histopathological findings showed dermal infiltrate of histiocytes with foamy macrophages and touton giant cells. We diagnosed this patient as adult onset eruptive xanthogranuloma. Our case is an exceptionally rare presentation of eruptive multiple xanthogranuloma in an adult with no associated disease. To our knowledge, this is the 3rd reported case of eruptive adult xathogranuloma with more than 20 lesions in Korean literature.

성인 황색육아종의 임상, 병리조직 및 면역조직화학적 연구

곽현빈 ( Hyun-bin Kwak ),정의성 ( Eui Sung Jung ),박상우 ( Sang-woo Park ),윤석권 ( Seok-kweon Yun ) 대한피부과학회 2019 大韓皮膚科學會誌 Vol.57 No.5

Background: Juvenile xanthogranuloma is a benign, self-limited disorder that usually occurs in infants and young children. Xanthogranuloma is rare in adults, and therefore studies reporting adult xanthogranuloma are limited. Objective: We investigated the clinical, histopathological, and immunohistochemical characteristics of adult xanthogranuloma. Methods: In this study, we evaluated 20 lesions in 19 patients with adult xanthogranuloma. Results: A male predominance was observed (male:female ratio 1.4:1), and the mean age of patients was 35.1±16.3 years (range 15∼66 years), with the peak incidence observed in patients in their 20s. Notably, 65.0% of the lesions developed on the head and neck. The nodular form was more common than the papular form of this condition. Histopathological examination revealed dense monomorphic histiocytic infiltration without lipidization and scattered eosinophils without multinuclear giant cells in 5 lesions (25.0%), foamy histiocytic infiltration with variations of completely developed Touton giant cells in 10 lesions (50.0%), and fibrohistiocytic proliferation in 3 lesions (15.0%). On immunohistochemical examination, histiocytes including giant cells showed positive test results with Factor XIIIa (90.9%), vimentin (100%), and CD68 (100%) and negative test results with CD1a, smooth muscle actin, and S-100 protein stains. Tumor excision was the treatment for choice. Conclusion: Adult xanthogranuloma most commonly manifested as the nodular form of the disease on the head and neck of men in their late 20s. Histopathologically, the classic Touton cell-rich stage was most commonly observed, followed by the stage of early predominantly mononuclear infiltration. This was a single-center, small-sized retrospective study; however, we expect the results of this study to contribute to a better understanding of adult xanthogranuloma. (Korean J Dermatol 2019;57(5):243∼250)

Adult xanthogranuloma: A clinical, pathological, and immunohistochemical study of 19 Korean cases

( Sang-woo Park ),( Eui-sung Jung ),( Hyun-bin Kwak ),( Soo-kyung Park ),( Kyung-hwa Nam ),( Jin Park ),( Seok-kweon Yun ),( Han-uk Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.2

Background: Juvenile xanthogranuloma is a benign, self-limited disorder that usually occurs in infants and young children. However, it is rare in adults and there is a lack of study on adult xanthogranuloma. Objectives: To investigate the clinical, pathological, and immunohistochemical characteristics of adult xanthogranuloma. Methods: This study included 20 lesions from 19 patients with adult xanthogranuloma. Results: There was a male predilection (M:F=1.4:1), and mean age was 35.1±16.3 years (range 15~66 years), with the peak in the twenties. 65.0% of the lesions developed on the head and neck. The nodular form was more common than the papular form. Histological examination in 5 lesions (25.0%) revealed dense monomorphic histiocytic infiltration without lipidization and scattered eosinophils. There was also absence of multinuclear giant cells. 10 lesions (50.0%) had foamy histiocytic infiltration with variations of fully developed Touton giant cells. 3 lesions (15.0%) showed fibrohistiocytic proliferation. Histiocytes, including giant cells, were generally positive for FXIIIa (90.9%), vimentin (100%) and CD68 (100%). The CD1a, SMA and S-100 protein reactions were all negative. Tumor excision was the treatment for choice. Conclusion: This retrospective study has a small sample size and is limited to a single center, but we expect the results of this study to contribute to better understanding adult xanthogranuloma.

소아에서 발생한 단발성 방추형 황색육아종

이중선 ( Joong Sun Lee ),이혜경 ( Hye Kyung Lee ),구대원 ( Dae Won Koo ) 대한피부과학회 2011 대한피부과학회지 Vol.49 No.7

Non-Langerhans cell histiocytoses are classified according to the predominant mononuclear (vacuolated, spindle-shaped, xanthomatized, scalloped, and oncocytic) and/or multinucleate (Touton, ground-glass appearance, Langhans, and foreign body) histiocytic cell types. Spindle-shaped histiocytes are evident in spindle cell xanthogranuloma and progressive nodular histiocytosis. Clinically, a single brown-yellow papule or nodule is characteristic of solitary spindle cell xanthogranuloma. Solitary spindle cell xanthogranuloma occurs with decreasing frequency on the head, neck, upper trunk, and occasionally the extremities of young adults (20~40 years of age). Although there have been a few reports of spindle cell xanthogranuloma, there has only been one case of spindle cell xanthogranuloma described in the Korean literature. We report a rare case of solitary spindle cell xanthogranuloma that developed in childhood and suggest this be included in the differential diagnosis among spindle cell tumors.

성인에서의 외이도 황색 육아종 1례

유동준(Dong Joon Yoo),이성훈(Sung-Hun Lee),여상원(Sang Won Yeo),박시내(Shi-Nae Park) 대한두개저학회 2014 대한두개저학회지 Vol.9 No.1

Xanthogranuloma is one of non-Langerhans cell histocytic disorders. And adult-onset huge xanthogranuloma of the external auditory canal is very rarely reported, especially on this anatomical site. We introduce a case of adult-onset huge xanthogranuloma of the external auditory canal. An 32-year-old female presented with intermittent left bloody otorrhea for two months. Radiological studies showed a solitary mass on the posterior wall of external auditory canal that had no invasion or erosion of temporal bone. The mass that originated from the posterior wall of the external auditory canal was excised and the defect site was reconstructed with ipsilateral superficial temporalis fascia. Pathologically, this huge mass was diagnosed as xanthogranuloma. We report this case as a very rare case of adult-onset xanthogranuloma in the external auditory canal as well as its excellent long-term result of surgery with the review of the literatures.

A case of juvenile xanthogranuloma of the nasal cavity

( Ju Wang Jang ),( Dong Uk Cheon ),( Sung Soo Han ),( Hyun-min Seo ),( Joung Soo Kim ),( Hee Joon Yu ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Juvenile xanthogranuloma is a benign, self-limited, non-Langerhans-cell histiocytosis that usually occurs in infancy and childhood. It generally develops in the head and neck but rarely in the nasal cavity. A 7-year-old girl presented with a 0.8x0.8 cm sized solitary yellowish nodule at the septum of the left nostril which has lasted for 3 months. The nodule had gradually increased in size without any subjective symptoms. The excisional biopsy revealed a mixed cellular infiltration composed of fibroblasts, histiocytes and multinucleated Touton giant cells in the dermis. Immunohistochemical stains were positive for CD68 and negative for CD1a. Based on these clinicopathological findings, the patient was diagnosed with juvenile xanthogranuloma. Patient showed no evidence of recurrence for 3 months. Herein, we report a case of juvenile xanthogranuloma of the nasal cavity, which has rarely been reported in the dermatologic literatures.

Solitary juvenile xanthogranuloma: clinical and dermoscopic findings

( Dae-lyong Ha ),( Tae-wook Kim ),( Sung-min Park ),( Hyun-joo Lee ),( Hyunju Jin ),( Hyang-suk You ),( Woo-haing Shim ),( Gun-wook Kim ),( Hoon-soo Kim ),( Hyun-chang Ko ),( Byung-soo Kim ),( Moon-bu 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: Juvenile xanthogranuloma (JXG) is a common non-Langerhans cell histiocytosis. JXG can have 2 main clinical forms with a popular form as multiple papules in infants and children and a nodular form as 1 or a few lesions in adults. Most data about JXG have been focused on common popular form, and clinical data about nodular form or solitary JXG are very limited. Objectives: To investigate the clinical and dermoscopic findings of solitary JXG. Methods: We retrospectively reviewed medical records, and clinical and dermoscopic photos of 51 patients with solitary JXG among 93 JXG patients confirmed histopathologically in the Pusan National University Hospital (Busan and Yangsan) from 2005 to 2017. Results: Solitary JXG was more common than classic multiple popular form (51/93, 54.8%). Of 51 patients with solitary JXG, 40 (78.4%) were children (range in 0.25-6) and 11 (21.6%) were adults (range in 22-55). Childhood JXG was bigger than adult JXG (mean diameter: 6.3 mm vs 3.7 mm, P < 0.0001). The predominant involved anatomical site was scalp (35%) in childhood JXG and face (36.4%) in adult JXG. Setting sun appearance was the most common dermoscopic feature. 23 lesions were removed during biopsy and 4 with follow-up had spontaneous involution. Conclusion: The majority of JXG studies were done on Caucasians and there has been no study focusing on solitary JXG like this study.

[P101] Plaque-type juvenile xanthogranuloma

( Young In Lee ),( Sang Ho Oh ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytosis (NLCH) occurring mostly in early childhood. It accounts for 80-90% of cases of NLCH. It is a benign, self-healing disorder, characterized by asymptomatic, yellowish papulonodular lesions. The symmetrical plaque-type JXG on face is a rare variant, that was first described by Gunson et al in 2008. Currently, only two case reports on facial symmetrical plaque-type JXG are recorded in literatures. In both cases, lesions lasted for longer than 6 years without a sign of spontaneous resolution, and no involvement of internal organs was noted. A 3-year-old Korean boy was referred to our clinic with bilateral yellowish indurated plaques on both cheeks. The asymptomatic plaques persisted for 1.5 years and had been slowly growing in size. Physical examination and history taking showed no ophthalmic or oral mucosal involvement, and palpable lymphadenopathy was not noticed during the exam. Laboratory findings including the lipid profiles showed the normal range. A skin biopsy was performed on the yellowish plaque from the right cheek, and the pathological findings were shown as JXG. Furthermore, CD68 was positive in histiocytes, whereas S-100 was negative by immunohistochemistry, therefore consistent with JXG. Hence, the patient was diagnosed with plaque-type juvenile xanthogranuloma.

성인 천식 동반 눈주변 황색육아종 1예

남승완(Seung Wan Nam),이주향(Ju Hyang Lee),우경인(Kyung In Woo),김윤덕(Yoon Duck Kim) 대한안과학회 2016 대한안과학회지 Vol.57 No.9

목적: 성인에서 안와를 침범하는 황색육아종은 드문 질환군으로 그중에서도 더욱 낮은 발생 빈도를 가지는 성인 천식 동반 눈주변 황색육아종 1예를 보고하고자 한다. 증례요약: 75세 남자가 15년 전부터 발생한 양측 눈꺼풀 종괴 및 노란색 피부 침착을 주소로 내원하였다. 13년 전 천식을 진단 받았다. 양안에서 노란색 피부 침착을 동반한 눈꺼풀 종괴 및 눈꺼풀처짐 소견을 보였고, 우안에서 더 심했다. 우안 안구돌출과 안구운동검사에서 안구운동의 제한 및 외사시, 상사시가 관찰되었다. 안와자기공명영상 검사에서 양측 눈꺼풀, 눈물샘, 부비동에 경계가 불분명한 종괴가 관찰되었으며, 우안은 안구 뒤까지 침윤성 종괴가 있었고, 외안근 비대가 관찰되었다. 좌측 위눈꺼풀수술 후 경구 azathioprine과 스테로이드 병합치료를 시작하였으며, 이후 눈꺼풀 종괴의 크기가 감소하였다. 이후 우측 위눈꺼풀수술 및 아래눈꺼풀수술을 추가로 시행하였다. 결론: 성인 천식 동반 눈주변 황색육아종은 재발이 쉽고 치료가 어려운 것으로 알려져 있으나, 수술적 치료와 함께 면역치료제-스테로이드 병합치료로 미용적, 기능적으로 우수한 결과를 얻을 수 있었다. Purpose: Adult xanthogranulomatous disease of the orbit including adult-onset asthma and periocular xanthogranuloma is rare. To the best of the author’s knowledge, adult-onset asthma and periocular xanthogranuloma have not been previously reported in Korea. Case summary: A 75-year-old man presented with a 15-year history of progressive bilateral yellowish eyelid mass. He had a history of adult-onset asthma diagnosed 13 years ago. On ocular examination, there were firm yellowish bilateral eyelid masses and ptosis markedly on the right eye. Exophthalmos was noted in the right eye. There were exotropia, hypertropia, and gaze restriction of the right eye. Orbit magnetic resonance imaging demonstrated an ill-defined bilateral infiltrative orbital mass extending to the lid, lacrimal gland, and sinuses. A right orbital mass extended to the posterior orbit. Enlargement of extraocular muscles was also noted in the right eye. Left upper eyelid mass debulking surgery was performed. A combination therapy of azathioprine and prednisolone was started. Mass debulking surgeries of the right upper and lower eyelids were performed. Conclusions: Adult-onset asthma and periocular xanthogranuloma is known as a refractory disease. Combination therapy with azathioprine and steroid therapy with mass debulking surgery is a good treatment option for adult-onset asthma and periocular xanthogranuloma.