Sirolimus—A targeted therapy for Rosai‐Dorfman disease

Golwala, Zainab; Taur, Prasad; Pandrowala, Ambreen; Chandak, Shraddha; Desai, Mukesh

doi:10.1002/pbc.27994

Cited by 5 publications

(4 citation statements)

References 17 publications

(29 reference statements)

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“…[13][14][15] Sirolimus is an understudied option for the treatment of RDD. 16 Cooper et al 17 first reported the use and efficacy of sirolimus in the treatment of systemic RDD with disease unresponsive to multiple treatments. This case was followed by two case reports of the efficacy of this calcineurin inhibitor in RDD lymphadenopathy, 16 although the efficacy of sirolimus in purely cutaneous RDD has not been previously reported.…”

Section: Discussionmentioning

confidence: 99%

“…Sirolimus inhibits possible deregulation of the mTOR pathway, thereby impairing the normal development of histiocyte progenitor cells, resulting in a reduction of these cells. 16 Understanding the pathogenesis of histiocytosis and its clinical manifestations will allow therapeutic guidelines to be developed to reduce the consequences and severity of these diseases.…”

Section: Discussionmentioning

confidence: 99%

“…Sirolimus is an understudied option for the treatment of RDD 16 . Cooper et al 17 first reported the use and efficacy of sirolimus in the treatment of systemic RDD with disease unresponsive to multiple treatments.…”

Section: Discussionmentioning

confidence: 99%

“…Sirolimus inhibits possible deregulation of the mTOR pathway, thereby impairing the normal development of histiocyte progenitor cells, resulting in a reduction of these cells 16 …”

Section: Discussionmentioning

confidence: 99%

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Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Tirado‐Sánchez

2023

Skin Health and Disease

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Sinus histiocytosis or Rosai‐Dorfman disease (RDD) is a rare disorder with severe lymphadenopathy and a limited clinical course, the aetiology of which is still controversial. The disease usually affects cervical nodes, with fever, polyclonal gammopathy, and leucocytosis with neutrophilia. Pure cutaneous involvement occurs as the only manifestation in only 3% of cases. Cutaneous RDD is often associated with infections, immunodeficiency, and autoimmune disorders. A 52‐year‐old patient presented with disseminated, recurrent, and relapsed pure cutaneous RDD that responded well to treatment with sirolimus and local infiltrations of methylprednisolone. The patient had multiple nonpainful nodular and tumour‐like lesions, histiocytic infiltrates with emperipolesis were observed on histologic examination, and positive immunohistochemistry for CD68, and S100. There is no standardised treatment, then the patient was treated with various therapies, including systemic steroids, chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), radiotherapy, and other immunosuppressive treatments. Some lesions were treated surgically, resulting in recurrence. Sirolimus and local infiltration with methylprednisolone were tried as salvage treatments, the patient responded well, reducing the incidence of new lesions during follow‐up, and the size of the preexisting lesions.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Sirolimus inhibits possible deregulation of the mTOR pathway, thereby impairing the normal development of histiocyte progenitor cells, resulting in a reduction of these cells 16 …”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Tirado‐Sánchez

2023

Skin Health and Disease

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Advances in our understanding of genetic markers and targeted therapies for pediatric LCH

Bahabri,

Abla

2024

Expert Review of Hematology

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Treatment of purely cutaneous Rosai–Dorfman disease with topical rapamycin

Balestri¹,

Magnano²,

Pedrolli³

et al. 2023

Clinical and Experimental Dermatology

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Purely cutaneous Rosai-Dorfman disease is a self-limiting and benign histiocytosis; however, some patients may be concerned about the aesthetic appearance, with a negative effect on quality of life. Even though many treatments have been proposed, no specific therapies are available and there is insufficient evidence supporting the superiority of any particular approach. Here we report the improvement of purely cutaneous Rosai-Dorfman disease with topical rapamycin.

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Sirolimus—A targeted therapy for Rosai‐Dorfman disease

Cited by 5 publications

References 17 publications

Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Advances in our understanding of genetic markers and targeted therapies for pediatric LCH

Treatment of purely cutaneous Rosai–Dorfman disease with topical rapamycin

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Sirolimus—A targeted therapy for Rosai‐Dorfman disease

Cited by 5 publications

References 17 publications

Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Recalcitrant primary cutaneous Rosai‐Dorfman disease. Efficacy of sirolimus and intralesional methylprednisolone

Advances in our understanding of genetic markers and targeted therapies for pediatric LCH

Treatment of purely cutaneous ​Rosai–Dorfman disease with topical rapamycin

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Product

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Treatment of purely cutaneous Rosai–Dorfman disease with topical rapamycin