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Histiocytosis and Lipid Storage Diseases
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Salwa Shabbir Sheikh, David F. Garvin
a. Juvenile Xanthogranuloma. Juvenile xanthogranuloma usually presents during infancy, but lesions may be present at birth or may appear during adulthood. There are widespread skin and mucous membrane nodules (Fig. 1) measuring 0.5–1.0 cm in diameter, which are yellow to red in color and most commonly affect the scalp and face, but may involve mesentery and viscera as well. The disease usually involutes spontaneously.
Benign tumors
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Juvenile xanthogranuloma (JXG) is a benign proliferative disorder mostly seen in young children as a yellow to reddish-tan, painless, dome-shaped nodule in the face and on the trunk. Subungual xanthogranuloma is very rare.275 It causes elevation and deformation of the nail plate. The clinical diagnosis, among others, concerns Spitz’s nevus.
Histiocytic lesions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Histopathology of juvenile xanthogranuloma is characteristic. There is a granuloma-like infiltrate composed of lymphocytes, some eosinophils, foam cells, and giant cells both of foreign and Touton type. The latter typically exhibit a central homogeneously eosinophilic cytoplasm, a wreath of nuclei, and an outer ring of foamy cytoplasm. Cell atypias are not seen. An occasional mitosis may be present.
Adult xanthogranulomatous disease of the orbit: case report of spontaneous regression and review of treatment modalities
Published in Orbit, 2020
Michelle M. Maeng, Kyle J. Godfrey, Sanjai Jalaj, Michael Kazim
Published results demonstrate varying degrees of success with surgical debulking. Shimizu et al. described a case of orbital xanthogranuloma without signs of regrowth at two years of follow-up after tumour resection without measurable excision margins.6 Sivak-Callcott et al. found that surgery alone was effective for six out of eight patients diagnosed with AAPOX and one patient with NXG.4 Ebrahimi et al. also described two cases of orbital xanthogranuloma without regrowth following complete resection but acknowledged that complete resection is generally impractical due to the infiltrative nature of the lesions.7 Despite several favourable reports, the majority of AOXGD cases recur following surgical debulking alone.1,2,5,8–10 Kiralti et al. advocated debulking orbital lesions combined with intravenous pulse methylprednisolone followed by at least six months of oral prednisolone for patients with AOX both with and without asthma. Using this approach, the authors reported complete clinical resolution in six adult patients by eight months and radiologic resolution by 18 months. No recurrence was reported with an average follow-up period of 50 months.11
Multiple subcutaneous xanthogranuloma at juxta-articular sites with bone cystic changes resembling rheumatoid arthritis: A case report
Published in Modern Rheumatology, 2018
Shoichi Kaneshiro, Kenrin Shi, Kosuke Ebina, Masao Yukioka, Hideki Yoshikawa, Norikazu Murata
The pathogenesis of xanthogranuloma is still unknown. Although phagocytosis of lipids by histiocytes/macrophages is a typical histopathologic finding, serum lipid profiles are usually normal in patients with both juvenile and adult form of xanthogranuloma [5], and they were also within normal range in our case. In our case as well as other reported cases with joint involvement, abnormalities in connective-tissue metabolism might have been underlying the pathogenesis, and a notion of “lipoid dermato-arthritis”, the term described by Warin et al. [13], would be introduced. Spontaneous resolution does not occur in adult form, whereas juvenile form usually regresses spontaneously within a year [15].
Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence
Published in Orbit, 2022
Aleza A Andron, Akshay Gopinathan Nair, David Della Rocca, Robert C Della Rocca, Harsha S Reddy
There are few reports in the literature pointing out the possible association between xanthogranuloma and IgG4-ROD.18–22 Singh et al. published a case where a patient with bilateral xanthelasmas developed an inflammatory syndrome with proptosis and ptosis.19 Biopsy was performed and histology revealed a xanthomatous inflammatory infiltrate. In addition, there was a dense population of IgG4 cells on histology with an IgG4/IgG ratio of 80% – fulfilling the histologic criteria of IgG4-ROD. They concluded that their patient had sclerosing IgG4-ROD with a xanthogranulomatous component. Muhar et al. published a series in which two patients with orbital NBX were found to have elevated IgG4 levels, and one patient manifesting with systemic disease.20 London et al. published a case series suggesting a link between AAPOX and IgG4-ROD.21 Two out of the three cases of confirmed AAPOX had both serology and histology confirming IgG4-ROD. They suggested a link between the two conditions. Verdijk et al. reported a retrospective case report of all confirmed cases of xanthogranuloma.18 In their report, 16 cases were evaluated clinically and specimens were re-stained, and 50% of the cases were found to fulfill the histologic criteria of IgG4-ROD. Only two were found to have suspected systemic involvement. In this study serology of the patients was not measured, which gives only partial positivity for IgG4-ROD. They concluded that despite the elevated levels of IgG4-ROD in these patients, most often it is not related to systemic manifestation of the disease. It seems that there exists an association between these two entities – but it is difficult to ascertain the exact nature of the relation. These diseases are similar in that they both can present with an orbital or adrenal lesion and also have systemic manifestations. Both can be associated with asthma and allergies and can be multifocal in the body. Serologically, they both can present with raised IgE levels as well as hypergammaglobulinemia. Our case had xanthogranulomatous disease subtype AOX, with no other systemic manifestations. The patient also fulfilled the criteria of IgG4-ROD both on serology and histopathology. Although he had no signs of inflammatory acute orbital disease or other signs of systemic disease, he was treated with steroids with reduction in the size of the mass and no further worsening.