Enfermedad de Rosai-Dorfman con presentación cutánea y ausencia de mutaciones BRAF-V600, KRAS y NRAS: ¿trastorno neoplásico o reactivo

Machado, Isidro; Fernández-Coronado, Javier Alcácer; Requena, Celia; Traves, Víctor; Martínez, Nuria Latorre; Ortega, Jose; Requena, Luis; García, Jorge Alberto Osuna

doi:10.1016/j.patol.2019.03.007

Cited by 1 publication

(3 citation statements)

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“…Emperipolesis may also occur in malignant histiocytosis. 10 In the absence of visceral involvement, therapeutic options depend on the impact of the skin lesions and include intralesional corticosteroid injections (isolated TIRADO-SÁNCHEZ -3 of 5 lesions), 12 cryotherapy, radiation therapy, and surgery. For refractory or extensive disease, high-dose thalidomide, oral dapsone, low-dose methotrexate, oral retinoids (isotretinoin, acitretin), imatinib, and systemic steroids are used.…”

Section: Discussionmentioning

confidence: 99%

“…8 Chronic infectious diseases such as cutaneous tuberculosis, leprosy and leishmaniasis can be distinguished from ERD by the presence of caseating granulomas, the presence of vacuolated macrophages (Virchow cells) and extensive inflammatory infiltration with the presence of plasma and amastigote cells within histiocytes. 10 In cases where diagnosis is difficult, PCR studies are required to identify the causative agent. Sarcoidosis can be distinguished from ERD by the presence of noncaseating granulomas on histopathology.…”

Section: Discussionmentioning

confidence: 99%

“…In Langerhans cell histiocytosis, it may manifest clinically as ERD, with S100 protein expressed in both diseases, although CD1A and Langerine are generally negative in ERD. Emperipolesis may also occur in malignant histiocytosis 10 …”

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%