Juvenile Xanthogranuloma and Other Non‐Langerhans Cell Histiocytoses

Ratzinger, Gudrun; Zelger, Bernhard

doi:10.1002/9781119142812.ch91

Cited by 2 publications

(5 citation statements)

References 227 publications

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“… 6 Almost always limited to the skin. 2 Dermoscopy Setting sun appearance 17 Limited data or report Histopathology Early lesions: Accumulation of histiocytes with no lipid infiltration and few lymphocytes and eosinophils. Mature lesions: Foam cells, Touton giant cells, histiocytes, lymphocytes, and eosinophils can be present.…”

Section: Discussionmentioning

confidence: 99%

“…Histiocytoses are a rare disorder characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues. 1 , 2 In a recent update published by the Histiocyte Society, 3 the Langerhans and non-Langerhans dichotomy has been questioned due to frequent overlaps of their clinical manifestations in both groups. As a result, they introduced a new classification consist of 5 groups of diseases: Langerhans-related (L), cutaneous and mucocutaneous (C), malignant histiocytoses (M), Rosai-Dorfman disease (R), as well as hemophagocytic lymphohistiocytosis and macrophage activation syndrome (H).…”

Section: Introductionmentioning

confidence: 99%

“… 5 Both JXG and BCH are characterized by yellowish-red papules or nodules on the face, neck, and trunk. 1 , 2 , 6 The clinical and histopathological features of these diseases are almost indistinguishable and may be overlapping. 7–9 Differences in histopathological features include the presence of Touton giant cells in JXG, while histiocyte infiltration can be found in both diseases.…”

Section: Introductionmentioning

confidence: 99%

“…Therapeutic options may include low-dose radiotherapy, intralesional steroid injections, cryotherapy, CO 2 laser treatment, and surgical excision. 1 , 2 , 6 At present, there is only one case report that utilizes non-invasive treatment for BCH using topical rapamycin that leads to a significant reduction in skin lesions. 13 Unfortunately, there is no data for JXG case treated with topical rapamycin.…”

Section: Introductionmentioning

confidence: 99%

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Successful Treatment of Non-Langerhans Cell Histiocytosis With Topical Rapamycin in Two Pediatric Cases

Effendi¹,

Rizqandaru²,

Yuliasari³

et al. 2022

CCID

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Non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by the proliferation of histiocytes in tissues that is excluded from the diagnostic criteria for LCH. Juvenile xanthogranuloma (JXG) and benign cephalic histiocytosis (BCH) are the most common types of cutaneous non-LCH. These two diseases share similarities in both clinical and histological features, therefore, they can be difficult to differentiate. Thorough physical, dermoscopic, and histopathological examinations are required to distinguish between JXG and BCH. We hereby present two rare cases of non-LCH in pediatric patients, presented with JXG and BCH. The dermoscopic examination of both cases showed a setting-sun appearance, while the histopathological examination revealed Touton giant cells in the JXG case, and massive lymphocyte infiltration in the BCH case. Both patients were treated with 1% topical rapamycin in a split-side comparison for the first 12 weeks, followed by applications on both sides for a total duration of 24 weeks. As a result, there was a significant reduction in the size of the lesion, leading to patient’s satisfaction. Rapamycin is an immunosuppressive agent with antineoplastic activity. Rapamycin can be used as an alternative non-invasive topical treatment option for JXG and BCH. However, long-term observations are required to assess its effectiveness and side effects.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Successful Treatment of Non-Langerhans Cell Histiocytosis With Topical Rapamycin in Two Pediatric Cases

Effendi¹,

Rizqandaru²,

Yuliasari³

et al. 2022

CCID

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“…Macrophages and dendritic cells are the major cell lines involved in histiocytosis. 3 The process begins as hematopoietic stem cells in the bone marrow progressively commit and develop into monocytes, one of the early responders to infection. As these monocytes migrate out of the bone marrow and travel to the bloodstream, their expression of surface markers CD14 and CD16 determine their classification into classical, non-classical, and intermediate types.…”

Section: Mononuclear Phagocytic Systemmentioning

confidence: 99%

Traversing the Spectrum of Non-Langerhans Cell Histiocytosis: A Case of Rosai-Dorfman Disease with Features of Necrobiotic Xanthogranuloma

Chen

David

et al. 2021

Acta Med Philipp

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Introduction. Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. Case. A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. Conclusion. Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

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Juvenile Xanthogranuloma and Other Non‐Langerhans Cell Histiocytoses

Cited by 2 publications

References 227 publications

Successful Treatment of Non-Langerhans Cell Histiocytosis With Topical Rapamycin in Two Pediatric Cases

Successful Treatment of Non-Langerhans Cell Histiocytosis With Topical Rapamycin in Two Pediatric Cases

Traversing the Spectrum of Non-Langerhans Cell Histiocytosis: A Case of Rosai-Dorfman Disease with Features of Necrobiotic Xanthogranuloma

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