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.

[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.

Giant congenital juvenile xanthogranuloma: Correct diagnosis is important to avoid unnecessary invasive

( Joon Ho Shim ),( Seung Hwan Oh ),( Ji Young Jun ),( Jun Hwan Kim ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Yoon Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis, Characterized by purple-red to yellow-brown papules or nodules, usually measuring less than 10 mm in diameter. Lesions larger than 20 mm in diameter are extremely rare and classified as giant JXG. Giant JXG, usually present at birth, are very uncommon lesions, and can often be misdiagnosed as congenital hemangioma or other malignant tumor. A healthy 2-month-old boy presented with a congenital skin colored asymptomatic plaque on left mandibular lesion, measured 20mm. A skin biopsy was performed on the lesion and the diagnosis of giant JXG was made. After 4 months, the tumor was enlarged to 32mm without sign of visceral involvement. In the case of cutaneous lesions without visceral involvement, the prognosis of giant JXG is excellent, and a ‘wait-and-see’ strategy is recommended. Spontaneous regression usually occurs within 6 months to 3 years. Correct diagnosis is important to avoid unnecessary excision or any aggressive procedure for this benign condition

Juvenile xanthogranuloma: A case of atypical manifestation

( Jungyoon Ohn ),( Yun Seon Choe ),( Kyu Han Kim ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

A 7-month-old male patient presented with a light brown colored bean-sized macule on the lower back from birth. It had multiple satellite lesions. Her medical history was non-specific. Histological findings showed foamy histiocytic collection in dermis with many multinucleated giant cells. Based on the clinical and histopathologic manifestation, she was diagnosed as having a juvenile xanthogranuloma. Juvenile xanthogranuloma, a variant of non-Langerhans’ cell histiocytosis, is characterized by yellow, red or brown colored, firm to rubbery, papules or nodules in infants. Typical manifestations would help make an easy diagnosis. But atypical cases were also reported, mimicking dermatofibroma, keloid, pyogenic granuloma, keratoacanthoma, and so on. In this case, it was presented as a nevus lipomatosus superficialis or connective tissue nevi.

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.

Symmetrical Giant Facial Plaque-Type Juvenile Xanthogranuloma: A Case Report with a Successful Response to Fractional CO₂ Laser Treatment

( Young In Lee ),( Sang Ho Oh ) 대한피부과학회 2019 Annals of Dermatology Vol.31 No.2

Symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) is a rare variant of juvenile xanthogranuloma, reported only in two cases in the literature. We report a case of a 3-year-old Korean boy who developed bilateral yellowish indurated plaques on both cheeks since 1 year after birth. A skin biopsy revealed numerous foam cells and Touton type giant cells throughout the upper dermis, and its immunohistochemical studies resulted positive for CD68 and negative for S-100. The boy was therefore diagnosed as a persistent SGFP-JXG. As the lesion did not show any signs of spontaneous regression, we performed a single session of fractional ablative CO₂ laser, which resulted in a significant reduction of the lesion. This is the first case report of a persistent SGFP-JXG on which a single ablative laser therapy was performed with a successful outcome. (Ann Dermatol 31(2) 209∼212, 2019)

눈물주머니에 발생한 연소형 황색육아종 1예

고상준,김상덕,Sang Jun Ko,Sang Duck Kim 대한안과학회 2012 대한안과학회지 Vol.53 No.2

Purpose: To report a rare case of juvenile xanthogranuloma in the lacrimal sac. Case summary: An 8-year-old female presented to our hospital with epiphora and palpable mass on the left medial canthal area. As a symptom, a sticky and bloody discharge through the punctum had begun on occasion 3 months previously. When pressure was applied on the left medial canthal area, a mucoid discharge through the punctum occurred, and a solid mass was palpable. The mass localized in the lacrimal sac and upper nasolacrimal duct was observed on orbit CT. No invasion into the surrounding tissue was present. The authors of the present study decided to perform endonasal dacryocystorhinostomy, and subsequently a skin-colored mass was found in the lacrimal sac. The histopathological and immunohistochemical diagnosis was histiocytic proliferation, compatible with juvenile xanthogranuloma. Six months later, the patient complained of epiphora recurrence with palpable mass on medial canthal area. The tumor recurrence was confirmed with enhanced orbit CT finding 14 months after the first surgery. A second surgery using endonasal dacryocystorhinostomy was performed to remove the mass. J Korean Ophthalmol Soc 2012;53(2):348-352

Is solitary benign cephalic histiocytosis early variant of juvenile xanthogranuloma?

( Ji Min Lee ),( Bo Young Kim ),( Chang Min Kim ),( Seung Hyun Chun ),( Sang Wook Son ),( Il Hwan Kim ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Benign cephalic histiocytosis (BCH) is a rare non-Langerhans histiocytosis, which manifests as asymptomatic orange-red papules mainly on the head and resolves in a few years. Solitary BCH has not been reported until now in our knowledge. The concept of non-Langerhans cell histiocytoses forming part of a spectrum of disease rather than being separate pathologic entities has been reported. Also, there are cases supporting that BCH is not a separate disease but a variant of juvenile xanthogranuloma (JXG). We report the case of solitary BCH, regarded as early variant of JXG. A 2-year-old girl presented with solitary erythematous to orange-colored papule on left cheek. Our first impression was a JXG. The histologic examination showed sufficient proliferation of large, epithelioid histiocytic cells with large cytoplasm within the upper and mid-dermis. Immunohistochemically, the histiocytes were positive for CD68 while lacking expression of CD1a and S-100. Therefore the patient was diagnosed as BCH. About 7 months after the first visit, the facial papule has not changed in size and color. Further research is needed on the relationship between BCH and JXG.

코피를 주소로 내원한 연소성 황색육아종

장혜빈,김나임,양형채 대한이비인후과학회 2021 대한이비인후과학회지 두경부외과학 Vol.64 No.2

Juvenile xanthogranuloma (JXG) is a rare benign histiocytic tumor, which was first describedby Adamson in 1905. JXG typically presents as solitary or multiple yellow-brown skin nodulesin infants, most often on the head, neck or upper trunk. Extracutaneous forms are rareand there are only several cases reported in the nasal cavity up to date. Previous cases werepresented with the mass effect such as nasal obstruction. We recently experienced a pediatricpatient with epistaxis and the nasal endoscopy suggested pyogenic granuloma. However, biopsyof the mass revealed JXG. Herein, we report a case of disseminated juvenile xanthomamisdiagnosed as pyogenic granuloma

거대 비지질성 연소성 황색 육아종

김진용 ( Jin Yong Kim ),윤소영 ( So Young Yoon ),최태현 ( Tae Hyun Choi ),조광현 ( Kwang Hyun Cho ) 대한피부과학회 2012 대한피부과학회지 Vol.50 No.3

Juvenile xanthogranuloma, which is the most common non-Langerhans cell histiocytosis of childhood, presents characteristic histopathologic features including abundant foamy macrophages and Touton giant cells. Generally, juvenile xanthogranuloma shows clinical features of less than 20 millimeters in size, butrarely, some lesions called giant juvenile xanthogranuloma exceed 20 millimeters. We report a case of a 5-month-old boy with solitary giant juvenile xanthogranuloma on his postauricular area with the histologic feature of nonlipidized form. The patient received simple excision. No evidence of local recurrence or systemic involvements have been observed during 4 months of follow up. (Korean J Dermatol 2012;50(3):238~242)