Cases reported "Xanthogranuloma, Juvenile"

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1/7. Verruciform xanthoma associated with squamous cell carcinoma.

    Verruciform xanthoma (VX) is a rare lesion of unknown etiology that is typically solitary and predominantly located within the oral cavity. Less commonly, they arise on the skin, with the majority of cases occurring in anogenital sites. They can be confused clinically with verruca vulgaris, condyloma, leukoplakia, verrucous carcinoma, and squamous cell carcinoma. Histologic features include acanthosis with uniform elongation of the rete ridges and xanthomatous cells that lie in and are typically confined to the papillary dermis. Although epidermal atypia is not a characteristic finding, we describe an unusual case of VX that has features of both VX and squamous cell carcinoma. In addition, there was a VX with typical histologic characteristics located at a separate site in the same patient. This case is also the first to our knowledge to be reported on the neck and axilla and is the third case associated with cutaneous graft versus host disease secondary to bone marrow transplant for acute lymphoblastic leukemia.
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2/7. association of juvenile xanthogranuloma with cafe-au-lait macules.

    A 2-year-old boy was referred to the dermatology services for the evaluation of yellowish-brown raised lesions over the face of 3 months' duration. In addition, he had multiple asymptomatic hyperpigmented spots over the trunk, which his parents had noticed at the time of birth. His physical and mental milestones of development were normal. Apart from the cutaneous lesions, the child was otherwise well and there was no history of seizures. He was the only child born to nonconsanguineous parents. No family history of similar lesions was available. The boy weighed 14 kg, had a height of 88 cm, and a head circumference of 48 cm. General physical and systemic examination was normal. Cutaneous examination revealed multiple, yellowish-brown papules over the forehead, sides of the face, and shoulders. The papules measured 0.5-0.8 cm in diameter, were discrete, well defined, oval and flat topped without any surface changes (Fig. 1). Multiple cafe-au-lait macules varying from 0.5 to 3.0 cm in diameter were present over the trunk (Fig. 2). There was no freckling in the axillary or the inguinal regions and no neurofibromas were present. A diagnosis of juvenile xanthogranuloma was considered. Complete blood counts, urine analysis, hepatic and renal function tests, and serum lipid levels were normal. A skin biopsy from a papule over the shoulder revealed a dense infiltrate in the dermis underlying a normal epidermis. The infiltrate was well demarcated and comprised lymphocytes, eosinophils, and foamy histiocytes along with Touton giant cells and proliferating fibroblasts. This confirmed the diagnosis of juvenile xanthogranuloma. The parents were informed about the benign and self-limiting nature of the disorder and were advised to bring the child for regular follow-up.
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3/7. Mitotically active juvenile xanthogranuloma.

    Juvenile xanthogranuloma is a histiocytic inflammatory disorder capable of presenting different histologic patterns. The classic form consists of sheets of foamy histiocytes and numerous multinucleated Touton-type giant cells containing no to very few mitotic figures. Nonlipidized juvenile xanthogranuloma consists of a diffuse monomorphic histiocytic infiltrate and usually shows a slightly higher mitotic index. Histologic hematoxylin-eosin staining techniques were used after excision of a pink ulcerated nodule in a 2-year-old girl. Histopathologic examination showed a nonencapsulated cellular proliferation mainly occupying dermis, but extending into the subcutaneous tissue as well. Most of the cells were epithelioid histiocytes, but foamy and Touton giant cells were also evident. The most striking feature was the presence of a high number of mitotic figures, as many as 23 per 10 high-power fields. We present an unusual case of classic juvenile xanthogranuloma with areas similar to those found in the nonlipidized type and with a high mitotic index.
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4/7. Langerhans cell histiocytosis and juvenile xanthogranuloma. Two case reports.

    BACKGROUND: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. OBSERVATIONS: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. RESULTS AND CONCLUSION: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or 'maturation' of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor.
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5/7. Juvenile xanthogranuloma presenting with unilateral prominent nodule of the eyelid: report of a case and clinicopathological findings.

    BACKGROUND: The purpose of this study is to report and describe the clinical and histopathologic findings in a patient with juvenile xanthogranuloma presenting as a relatively large, solitary, pedunculated mass on the left upper eyelid. CASE: A 31-month-old Japanese girl presented with a pedunculated nodular lesion that had been developing since 4 months earlier. Otherwise, no abnormal findings were identified in either eye. In addition, no other abnormalities, including skin eruptions or nodules, were detected anywhere. The lesion was completely excised and examined histopathologically. OBSERVATIONS: Microscopic study revealed a histiocytic proliferation accompanied by lymphocytes and occasional plasma cells and eosinophils within the dermis. A moderate number of giant cells of the foreign-body and Touton types were present. In the immunohistochemical analysis, the proliferative histiocytes and giant cells showed positive immunoreactivity for vimentin and macrophage markers and negativity for S-100 protein. The diagnosis of juvenile xanthogranuloma was made. The findings of further detailed ophthalmologic and systemic examinations and evaluations were unremarkable. Therefore, the diagnosis was made of juvenile xanthogranuloma, solitary type, occurring on the eyelid. CONCLUSIONS: Although juvenile xanthogranuloma of the eyelid is uncommon and its occurrence anywhere in the eye is rare, ophthalmologists should be familiar with this entity. Juvenile xanthogranuloma should be included in the differential diagnosis of eyelid mass lesions.
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6/7. Juvenile xanthogranuloma of temporal bone--a case report.

    Juvenile xanthogranuloma is an unusual condition of childhood. It commonly presents as cutaneous lesions normally localized to the dermis and is benign. A case report of a two-year-old girl with juvenile xanthogranuloma involving the right temporal bone (petro-mastoid region) which was locally destructive, is presented.
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7/7. Juvenile xanthogranuloma occurring in the oral cavity: case report and histopathological findings.

    Juvenile xanthogranuloma is commonly seen in the dermis, and only very rarely develops in the oral mucosa. Here were report a case that occurred in the anterior palate of a 9-year-old boy. The lesion appeared as a dark red and well-defined nodule measuring 12 x 14 mm. Histologically, it consisted of a proliferation of histiocytes and fibroblastic stroma intermingled with foamy cells. Many lipid droplets without limiting membrane were observed in the cytoplasm under electron microscopy, but no Langerhans' cell granules were observed. The proliferative histiocytes were positive for lysozyme and macrophage HAM56 under immunohistochemical observation, but not for S-100 protein. From these findings, the lesion was diagnosed as juvenile xanthogranuloma. The post-operative course, now amounting to 7 years, has been uneventful.
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