Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.
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PMID:Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma. 1604 46

A 3-year-old Thai boy suffered from two histiocytoses, Rosai-Dorfman disease (RDD) and juvenile xanthogranuloma (JXG). The patient first presented with massive cervical lymphadenopathy at the age of one year. Biopsy revealed typical RDD; abnormally large CD68- and S-100 protein-positive histiocytes with occasional emperipolesis filled up the sinuses. Two years later, he developed polyuria and polydypsia. Skull film demonstrated osteolytic lesions at the occiput and left parietal region. Enlargement of the pituitary stalk was found on the magnetic resonance imaging. Despite the clinical impression of Langerhans cell histiocytosis, biopsy of the occipital lesion disclosed numerous large histiocytes with foamy cytoplasm. Several Touton giant cells with wreath-like arrangement of the nuclei were also observed. The abnormal cells expressed CD68 and factor XIIIa, but were non-reactive with S-100 protein and CD1a. Biopsy of the pituitary stalk was not performed According to the authors' literature search, this represents the first report of RDD and JXG affecting the same person.
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PMID:Rosai-Dorfman disease and juvenile xanthogranuloma in a Thai boy: report of a case. 1658 88

NKI/C3 originally was described as a marker for melanoma. Recently, it resurfaced as a marker to separate cellular neurothekeoma from other dermal tumors in the differential diagnosis. To determine the sensitivity and specificity of NKI/C3, we evaluated its staining pattern in 709 normal and neoplastic tissues, including 92 dermal tumors, using tissue microarrays and conventional sections. We found that although NKI/C3 is positive in only a few normal tissues, it stains a broad spectrum of neoplastic tissues. NKI/C3 is also positive in many dermal tumors of possible histiocytic origin, including juvenile xanthogranuloma (6/10), atypical fibroxanthoma (4/12), cellular fibrous histiocytoma (5/10), reticulohistiocytoma (3/6), and xanthoma (8/10). However, it is negative in epithelioid cell histiocytomas (0/7) and Langerhans cell histiocytosis (0/9). Given the wide spectrum of positive staining in morphologic mimics of cellular neurothekeomas, pathologists should be cautious when making this diagnosis based solely on positive staining with NKI/C3.
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PMID:Frequent positive staining with NKI/C3 in normal and neoplastic tissues limits its usefulness in the diagnosis of cellular neurothekeoma. 1693 67

We present the case of a 10-week-old girl who had erythematous papules with a yellowish hue from birth with diagnosis of Langerhans cell histiocytosis, that was accompanied by a lytic lesion in the skull and hepatic involvement. After several months of treatment with prednisone and vinblastine with skin and systemic improvement, several rounded erythematous papules with a yellowish hue appeared in the right cheek. The biopsy showed a histiocytic infiltrate with positivity for CD68 and negative staining for S100 and CD1a, with a final diagnosis of juvenile xanthogranuloma. This association has been previously described in the literature in few cases. Although several hypotheses have been suggested, the causal relationship between both entities has still not been demonstrated.
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PMID:[Juvenile multiple xanthogranuloma in a patient with Langerhans cell histiocytosis]. 1717 66

Juvenile xanthogranuloma (JXG) is one of the most common non-Langerhans cell histiocytosis in children. Usually cutaneous, there are disseminated forms. However, neurological localization remains exceptional. A 7-month-old boy had been admitted for subdural effusion due to non-accidental head injury and skin nodular lesions. A biopsy of a skin lesion was considered suggestive of JXG. Skin, eyes, brain, lungs, liver, and testicles were involved. Systemic treatment of JXG was begun with vinblastine. It allowed the regression of skin, lung, and CNS lesions. At age of 11 years, he had not reappearance of the xanthogranuloma. This report emphasizes the possible presentation of xanthogranuloma with subdural effusions, the organs which should be examined in case of disseminated forms and the efficiency of vinblastin.
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PMID:Subdural effusion in a CNS involvement of systemic juvenile xanthogranuloma: a case report treated with vinblastin. 1772 Mar 45

Juvenile xanthogranuloma (JXG) is a benign, self-healing non-Langerhans cell histiocytosis of unknown aetiology, most commonly occurring in infants and children. JXG is characterized by solitary or multiple yellowish cutaneous nodules, the two common clinical variants being a small nodular form and large nodular form, which frequently coexist. Unusual morphological presentations include keratotic, lichenoid, pedunculated, subcutaneous, clustered, plaque-like and giant lesions. We describe the first reported case of 'disseminated' clustered juvenile xanthogranuloma presenting in an infant.
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PMID:Disseminated clustered juvenile xanthogranuloma: an unusual morphological variant of a common condition. 1847 8

Xanthogranuloma (XG) is a rare, non-Langerhans cell histiocytosis (LCH) that most commonly presents in infancy or early childhood. The condition is typified by the formation of reddish to yellow papules and nodules that are usually solitary. Xanthogranuloma rarely occurs in adults with immunohistochemical features similar to those seen in juvenile XG. Lesions in the adult form seen in juvenile XG. Lesions in the adult form also are typically solitary. We describe a 70-year-old white man who presented with widespread flat-topped, reddish to yellow papules and nodules with histologic and immunohistochemical findings consistent with XG. We explore the pathogenesis, differential diagnosis, prognosis, and treatment of this rare eruption. Comparison of adult and juvenile XG will facilitate a better understanding of the disease. Although rare, XG is an important disease to consider in the differential diagnosis of xanthomatous disease in adults.
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PMID:Cutaneous disseminated xanthogranuloma in an adult: case report and review of the literature. 1953 82

An 11-year old Caucasian female with a remote history of urticaria pigmentosa presented with a neck mass. A biopsy demonstrated a large intradermal nodule composed of unusually large epithelioid mast cells, including a prominent subset with bi-lobed and multi-lobed nuclei. By immunohistochemistry, the cells expressed CD117 (C-Kit), mast cell tryptase, CD68, and CD25, and were negative for CD163, CD1a, and S-100, confirming the diagnosis of mastocytoma. Equally prominent was an admixed infiltrate of CD68 and CD163-positive xanthomatous histiocytes that included Touton-type giant cells. Eosinophils were abundant. At 7 months follow-up, there was no recurrence of the lesion following complete excision. However, given the unusual cytologic features, close clinical observation is warranted, as the long-term biologic potential of mastocytoma with this degree of cytologic atypia is uncertain. Awareness of this unusual morphologic variant is also important as the histologic features may mimic such childhood neoplasms as juvenile xanthogranuloma and Langerhans cell histiocytosis.
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PMID:Histiocyte-rich pleomorphic mastocytoma: an uncommon variant mimicking juvenile xanthogranuloma and Langerhans cell histiocytosis. 1960 70

A 15-month-old boy was seen because of two distinct types of lesions, namely, yellowish papules on the scalp and face, and hemorrhagic macules and papules on the trunk. A biopsy specimen from one of the yellowish papules showed histopathologic and immunohistochemical changes of both juvenile xanthogranuloma and Langerhans cell histiocytosis. The section from the center of the biopsy specimen showed a proliferation of foamy histiocytes, among them Touton giant cells, which were positive for CD68, but negative for S-100 and CD1a. At the edges of the specimen was a predominantly histiocytic infiltration in the papillary dermis that was positive for S-100 and CD1a, but negative for CD68. The patient died 12 days after hospital admission consequent to disseminated intravascular coagulopathy. We did not biopsy the hemorrhagic lesions; however, this combination of findings suggests a possible relationship between juvenile xanthogranuloma and Langerhans cell histiocytosis, as previously reported.
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PMID:A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis. 1996 87

Juvenile xanthogranuloma in adulthood is an infrequent non-Langerhans cell histiocytosis, which may simulate malignant tumors such as basal cell carcinoma (BCC) or amelanotic melanoma. Dermoscopy has been described as a useful tool in the preoperative diagnosis of xanthogranuloma. We report a xanthogranuloma on the suprapubic area of a 48-year-old female, which clinically and dermoscopically mimicked a BCC with a yellowish hue and arborizing vessels. Reflectance confocal microscopy exhibited large highly refractive atypical cells in the dermis, some of them with pleomorphic nuclei, corresponding to Touton cells in the histopathological study. To our knowledge this is the first description of the clinical, dermoscopic and confocal microscopy correlations of a xanthogranuloma.
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PMID:Adult xanthogranuloma mimicking basal cell carcinoma: dermoscopy, reflectance confocal microscopy and pathological correlation. 2867 10


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