Juvenile Xanthogranuloma

Benign disorder of infants and children caused by proliferation of HISTIOCYTES, macrophages found in tissues. These histiocytes, usually lipid-laden non-Langerhans cells, form multiple yellow-red nodules most often in the skin, the eye, and sometimes in the viscera. Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis.
Also Known As:
Xanthogranuloma, Juvenile; Juvenile Xanthogranulomas; Juvenile Xanthoma; Juvenile Xanthomas; Nevoxanthoendotheliomas; Xanthogranulomas, Juvenile; Xanthomas, Juvenile; Nevoxanthoendothelioma; Xanthoma, Juvenile
Networked: 75 relevant articles (4 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)
2. Erdheim-Chester Disease
3. Benign Fibrous Histiocytoma (Dermatofibroma)
4. Malignant Fibrous Histiocytoma
5. Neoplasms (Cancer)


1. Shields, Carol L: 2 articles (01/2015 - 03/2008)
2. Tran, Don T: 2 articles (11/2009 - 10/2008)
3. Khoo, Chloe T L: 1 article (01/2015)
4. Samara, Wasim A: 1 article (01/2015)
5. Magrath, George: 1 article (01/2015)
6. Henry, Christopher R: 1 article (06/2014)
7. Murray, Timothy G: 1 article (06/2014)
8. Abbey, Ashkan M: 1 article (06/2014)
9. Berrocal, Audina M: 1 article (06/2014)
10. Ashkenazy, Noy: 1 article (06/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile Xanthogranuloma:
1. CladribineFDA LinkGeneric
2. Triamcinolone Acetonide (Azmacort)FDA LinkGeneric
3. Dermatofibrosarcoma protuberansIBA
02/01/1981 - "This study confirmed the histiocytic nature of juvenile xanthogranuloma and multicentric reticulohistiocytosis, supported the interpretation that there is a histiocytic component in the lesions of histiocytosis X, and cast some doubt on the alleged histiocytic nature of "fibrous" dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma."
02/01/1981 - "Histiocytosis X, multicentric reticulohistiocytosis, juvenile xanthogranuloma, the "fibrous" type of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma are all characterized by dermal and/or subcutaneous infiltrates composed at least partially of cells having morphologic features suggestive of histiocytes. "
07/01/2009 - "We evaluated PU.1 expression in 78 cases of primary skin neoplasms, including 9 reticulohistiocytomas, 9 Langerhans cell histiocytoses, 7 juvenile xanthogranulomas, 9 fibrous papules, 8 dermatofibromas, 12 dermatofibrosarcoma protuberans, 9 Spitz nevi, and 15 malignant melanomas. "
07/01/2004 - "In contrast all the following cases were completely unreactive: (i) epithelial tumours (seborrhoeic keratosis, squamous cell carcinoma, Bowen's disease, actinic keratosis, genital Paget's disease); (ii) follicular tumours (basal cell carcinoma, trichilemmoma, trichoepithelioma, trichoblastoma, keratoacanthoma, proliferating trichilemmal tumour, pilomatricoma); (iii) melanocytic tumours (malignant melanoma, naevus cell naevus, Spitz naevus, blue naevus); (iv) neural tumours (schwannoma, neurofibroma, Merkel cell neoplasm); (v) mesenchymal tumours (soft fibroma, dermatofibroma, dermatofibrosarcoma protuberans, vascular leiomyoma, leiomyosarcoma, lipoma, juvenile xanthogranuloma, angiomyoma); and (vi) other sweat gland tumours (poroid neoplasms, syringoma, cylindroma, clear cell hidradenoma, spiradenoma, syringoid eccrine carcinoma, mucinous carcinoma, apocrine cystadenoma, syringocystadenoma papilliferum, apocrine adenocarcinoma). "
02/01/1989 - "To detect LZ in these cells morphologically, I have done electron microscopic observations of the following skin diseases; sarcoidosis, lupus vulgaris, lupus miliaris disseminatus faciei (LMDF), tattoo granuloma, lichen nitidus, foreign body granuloma, granuloma annulare, xanthelasma, xanthoma tuberosum, xanthoma planum, juvenile xanthogranuloma, giant cell tumor of tendon sheath, dermatofibroma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, granulation tissue of burn, hypertrophic scar, and histiocytosis X. From both the immunohistochemical and the electron microscopic features it was concluded that a) immunohistochemically LZ-positive cells from lesions of sarcoidosis, lupus vulgaris, LMDF and tattoo granuloma had a number of electron-lucent bodies (ELB) or microvesicles in their cytoplasm, b) lichen nitidus and xanthoma tuberosum had few LZ-positive cells and the ELB were not observed, and c) the other diseases were LZ-negative, and the ELB were also absent. "
4. S100 Proteins (S 100 Protein)IBA
5. SteroidsIBA
6. Factor XIIIaIBA
7. AntigensIBA
03/01/2001 - "Juvenile xanthogranuloma: case report with immunohistochemical identification of early and late cytomegalovirus antigens."
12/01/1992 - "For example, the M phi s in cat-scratch fever are nearly unique in the expression of the "early inflammation" antigen identified by antibody 27E10, and the M phi s in juvenile xanthogranulomas, unlike those in most of the other conditions, lacked the antigen detected by antibody 25F9. "
12/01/1992 - "Using a large panel of monoclonal antibodies to monocyte/M phi-related antigens and a frozen-section immunoperoxidase technique, the following conditions were studied: granulomatous inflammation of unknown etiology, sarcoidosis, cat-scratch fever, toxoplasmosis, Gaucher's disease, and juvenile xanthogranulomas. "
06/01/2000 - "To explore the use of immunohistochemistry for this diagnostic problem, we examined the expression of S-100 protein, gp100 (the antigen recognized by HMB-45), tyrosinase (T311), Melan-A (A103), Factor XIIIa (FXIIIa), and CD68 in 10 juvenile xanthogranulomas (JXGs), five epithelioid histiocytomas (EHs), and 15 melanocytic nevi composed of large epithelioid cells. "
12/01/1987 - "Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. "
8. Muramidase (Lysozyme)IBA
9. Cholestanol (Coprostanol)IBA
10. CholesterolIBA

Therapies and Procedures

1. X-Ray Therapy
2. Radiotherapy
3. Drug Therapy (Chemotherapy)
4. Salvage Therapy
5. Curettage