Phenotypic Heterogeneity of the Dermal Monocyte/Macrophage System
Brian J. Nickoloff in Dermal Immune System, 2019
Histiocytosis X65 is a disease resulting from abnormal accumulation and proliferation of dendritic cells with the phenotype of cutaneous Langerhans cells.66 It has been proposed that proliferation and accumulation of these cells might result from a continuous and uncontrolled immunologic stimulation. Langerhans cell histiocytosis is difficult to distinguish from juvenile xanthogranuloma exhibiting similar histological changes. In our study, we were able to demonstrate the Ki-M1P−, S-100+ immunophenotype of Langerhans cell histiocytosis. This result underlines the concept of this entity being a Langerhans cell disease. In contrast, juvenile xanthogranuloma exhibits the inverse phenotype with regard to these two markers. Thus, immunophenotypical analysis with Ki-M1P and S-100 facilitates the differential diagnosis of these two diseases.
Histiocytosis and Lipid Storage Diseases
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
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.
Histiocytic lesions
Eckart Haneke in 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.
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.
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