Sinus Histiocytosis (Rosai-Dorfman Disease)

Benign, non-Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.

Also Known As:

Rosai-Dorfman Disease; Histiocytosis, Sinus; Sinus Histiocytosis with Massive Lymphadenopathy; Destombes Rosai Dorfman Syndrome; Disease, Rosai-Dorfman; Histiocytoses, Sinus; Rosai Dorfman Disease; Sinus Histiocytoses; Syndrome, Destombes-Rosai-Dorfman; Destombes-Rosai-Dorfman Syndrome

Networked: 279 relevant articles (19 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases: 6
- Lymphatic Diseases: 92
  - Histiocytosis: 520
    - Non-Langerhans-Cell Histiocytosis: 87
      - Sinus Histiocytosis: 279
        Asrar Facharzt Haque syndrome

Related Diseases

1.	Histiocytic Necrotizing Lymphadenitis (Kikuchi Disease)
2.	Lymphadenopathy
3.	Hodgkin Disease (Hodgkin's Disease)
4.	Non-Langerhans-Cell Histiocytosis (Histiocytosis, Non Langerhans Cell)
5.	Paraparesis

Experts

1.	Khoury, Joseph D: 3 articles (01/2022 - 01/2014)
2.	Sriha, Badreddine: 3 articles (10/2021 - 11/2007)
3.	Garces, Sofia: 2 articles (01/2022 - 01/2019)
4.	Li, Shaoying: 2 articles (01/2022 - 01/2019)
5.	Medeiros, L Jeffrey: 2 articles (01/2022 - 01/2019)
6.	Xu, Jie: 2 articles (01/2022 - 01/2019)
7.	Denguezli, Mohamed: 2 articles (10/2021 - 11/2007)
8.	Cohen-Aubart, Fleur: 2 articles (01/2021 - 01/2019)
9.	Haroche, Julien: 2 articles (01/2021 - 01/2019)
10.	Fujita, Shuichi: 2 articles (10/2020 - 04/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sinus Histiocytosis:

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2009 - "Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid." 05/01/2007 - "Usefulness of oral corticosteroid in Rosai-Dorfman disease." 03/01/2014 - "The diagnosis of Rosai-Dorfman disease is usually good, since 40 % of the patients present a spontaneous remission if they are treated with oral corticosteroids, even though the lesion can be managed with fractionated radiotherapy or with radical surgery. " 04/01/2009 - "We present herein a Japanese case of Rosai-Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. " 06/01/2008 - "We report the case of a 41-year-old female with bilateral orbital Rosai-Dorfman disease (RDD) and the result of corticosteroid therapy. "
2.	Thalidomide (Thalomid)FDA Link 12/01/2021 - "Long-time remission of laryngeal Rosai-Dorfman disease with thalidomide: a report of three cases." 05/01/2003 - "Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment." 04/01/2022 - "Thalidomide as treatment of refractory thoracic Rosai-Dorfman disease." 01/01/2019 - "Rosai-Dorfman disease successfully treated with thalidomide: A case report." 09/01/2016 - "Rosai-Dorfman disease successfully treated with thalidomide."
3.	SteroidsIBA 01/01/2020 - "Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai-Dorfman Disease." 07/01/2015 - "Intralesional steroid for orbital manifestations of Rosai-Dorfman disease." 11/01/2011 - "Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery." 06/01/2008 - "Extranodal Rosai-Dorfman disease with bilateral orbital involvement: Report of a case treated with systemic steroid alone." 09/01/2003 - "Response of cutaneous Rosai-Dorfman disease to topical and intralesional steroids."
4.	Methotrexate (Mexate)FDA LinkGeneric 11/01/2022 - "Cutaneous Rosai Dorfman disease harboring RET and MAP2K1 mutations, successfully treated with methotrexate." 12/24/2020 - "Intralesional methotrexate used in cutaneous Rosai-Dorfman disease." 10/01/2018 - "Low-dose weekly methotrexate used to treat cutaneous Rosai-Dorfman disease." 07/01/2014 - "Cutaneous Rosai-Dorfman disease successfully treated with low-dose methotrexate." 07/01/2012 - "To review the classic physical and histologic findings in Rosai-Dorfman disease and summarize the literature supporting the use of methotrexate in this condition. "
5.	1-phenyl-3,3-dimethyltriazene (PDT)IBA 09/01/2020 - "We present a case of cutaneous Rosai-Dorfman disease (CRDD) on the right cheek that responded well to combination of subtotal resection and ALA-PDT. " 09/01/2020 - "Successful treatment of large cutaneous facial Rosai-Dorfman disease using combination of subtotal resection and ALA-PDT: A case report." 03/01/2018 - "Successful treatment of Rosai-Dorfman disease using ALA-PDT." 09/01/2020 - "We believe that the combination of subtotal resection and ALA-PDT treatment for large cutaneous facial Rosai-Dorfman disease patients is a more effective, highly satisfying, and quick therapy worth promoting." 11/30/2023 - "Treatment of Cutaneous Rosai-Dorfman Disease with ALA-PDT Combined with Low-dose Oral Corticosteroids: A Case Report."
6.	Acitretin FDA Link 09/01/2010 - "The objective of this report is to illustrate that acitretin may be effective in treating cutaneous Rosai-Dorfman disease. " 09/01/2010 - "Our experience with a patient with a case of cutaneous Rosai-Dorfman disease persisting after surgical excision demonstrates that acitretin may be an effective therapeutic option for cutaneous Rosai-Dorfman disease." 09/01/2010 - "Cutaneous rosai-dorfman disease persisting after surgical excision: report of a case treated with acitretin." 11/01/2007 - "Extensive purely cutaneous Rosai-Dorfman disease responsive to acitretin." 09/01/2010 - "A patient with cutaneous Rosai-Dorfman disease refractory to antibiotics, steroids, and surgical excision was treated with acitretin for a period of four months and was closely monitored for improvement in the lesion. "
7.	Muramidase (Lysozyme)IBA 10/01/1999 - "Immunophenotypic studies, which showed a CD68+, lysozyme+, UCHL-1+, OPD4-, CD4-, s100- profile, were helpful in distinguishing these lesions from other processes, including Kikuchi-Fujimoto disease and Rosai-Dorfman disease. " 09/01/1987 - "Sinus cells in ordinary sinus histiocytosis were constantly positive for ACT and variably positive for lysozyme and were generally negative for S-100 protein. " 10/01/2023 - "Using immunohistochemistry, we arranged the 22 cases into 4 subtypes that correspond to the lines of differentiation from monocytic and dendritic cell precursors as follows: (1) macrophage (n = 5): CD68+, CD163+, CD14+, and Factor 13a+; (2) monocyte-macrophage (n = 5): CD68+, CD163+, CD14+, S100+, and OCT2+; (3) dendritic cell (n = 6): CD68+, CD11c+, S100+, lysozyme+, ZBTB46+, and CD1a/langerin < 5%; and (4) Langerhans cell (n = 6): CD68+, CD11c+, S100+, ZBTB46+, CD1a+, and langerin+. The phenotypic subtypes align with those seen in low-grade histiocytic neoplasms as follows: MH-macrophage type correlates with Erdheim-Chester disease phenotype; MH-monocyte-macrophage type with Rosai-Dorfman disease phenotype, and MH-Langerhans cell type with Langerhans cell histiocytosis. " 01/01/2002 - "Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. "
8.	Interferon-alpha (Interferon Alfa)IBA 06/02/2001 - "[Long term survival in a patient with aggressive Rosai-Dorfman disease treated with interferon alpha]." 05/01/1997 - "Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha." 12/01/1995 - "Long-term survival in a patient with Rosai-Dorfman disease treated with interferon-alpha." 02/03/1996 - "[Sinus histiocytosis of the Rosai-Dorfman type following Hodgkin disease: efficacy of interferon-alpha]."
9.	GlucocorticoidsIBA 01/01/2020 - "A diagnosis of extranodal Rosai-Dorfman disease was made, and the patient received systemic glucocorticoids, with considerable improvement after 4 months of therapy." 03/01/2021 - "A Case of Cutaneous Rosai-Dorfman Disease Treated by Intralesional Injections of Glucocorticoid."
10.	AcidsIBA 10/18/2023 - "Fractional laser combined with 5-Aminolevulinic acid photodynamic therapy for the treatment of Cutaneous Rosai-Dorfman Disease: A case report." 03/01/2022 - "5-Aminolevulinic acid photodynamic therapy combined with CO2 laser therapy for the treatment of cutaneous Rosai-Dorfman disease: A case report." 03/01/2022 - "We report the case of a patient diagnosed with cutaneous Rosai-Dorfman disease (CRDD) who was treated successfully with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) combined with carbon dioxide (CO2) laser therapy. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/1983 - "Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy." 01/01/2015 - "Histopathology of the lesion was reported as being consistent with Rosai-Dorfman disease and the patient is currently undergoing chemotherapy. " 01/01/1995 - "Evidence of responsiveness to chemotherapy in aggressive Rosai-Dorfman disease." 05/01/2006 - "Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. " 12/01/1998 - "This case demonstrates the potential efficacy of chemotherapy in the treatment of compressive optic neuropathy in cases of orbital sinus histiocytosis with massive lymphadenopathy."
2.	Therapeutics 03/01/2022 - "CRDD is a rare form of Rosai-Dorfman disease that has various clinical presentations and multiple possible therapies with varying efficacies. " 01/01/2022 - "Case report: BRAF-inhibitor therapy in BRAF-mutated primary CNS tumours including one case of BRAF-mutated Rosai-Dorfman disease." 07/10/2019 - "This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy." 01/01/1982 - "Sinus histiocytosis with massive lymphadenopathy (SHML) is generally regarded as a benign, self-limited, pseudolymphomatous process requiring little or no therapy. " 05/01/2006 - "Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. "
3.	Radiotherapy 05/01/2022 - "Treatment of multifocal cutaneous Rosai-Dorfman disease with radiotherapy." 10/27/2014 - "Extranodal rosai-dorfman disease of the bone treated with surgery and radiotherapy." 11/01/2012 - "Cutaneous Rosai-Dorfman disease of the right ear responsive to radiotherapy." 10/01/2008 - "A case of orbital Rosai-Dorfman disease responding to radiotherapy." 10/01/2001 - "Review of the literature on intracranial Rosai-Dorfman disease leads to the suggestion that postoperative radiotherapy may be advisable in all cases."
4.	Aftercare (After-Treatment) 08/01/2016 - "[Sinus histiocytosis with massive lymphadenopathy: FDG-PET/CT documented partial remission after treatment with 2-chlorodeoxyadenosine]." 09/01/2023 - "• Rosai-Dorfman disease usually has a high overall response rate after treatment." 04/01/2009 - "We present herein a Japanese case of Rosai-Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. " 12/01/2022 - "Outcomes After Treatment With Cobimetinib in Patients With Rosai-Dorfman Disease Based on KRAS and MEK Alteration Status."
5.	Photochemotherapy (Photodynamic Therapy) 10/01/2021 - "Regression of Intraocular Rosai- Dorfman Disease Following Treatment with Photodynamic Therapy." 10/18/2023 - "Fractional laser combined with 5-Aminolevulinic acid photodynamic therapy for the treatment of Cutaneous Rosai-Dorfman Disease: A case report." 03/01/2022 - "5-Aminolevulinic acid photodynamic therapy combined with CO2 laser therapy for the treatment of cutaneous Rosai-Dorfman disease: A case report." 03/01/2022 - "We report the case of a patient diagnosed with cutaneous Rosai-Dorfman disease (CRDD) who was treated successfully with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) combined with carbon dioxide (CO2) laser therapy. "