Successful treatment of refractory cutaneous Rosai–Dorfman disease with vincristine

Abstract

Dear Editor, We report a patient with refractory cutaneous Rosai-Dorfman disease (RDD) who was successfully treated with vincristine. A 41-year-old man presented with a 2-year history of slowly growing erythematous nodules and plaques on the bilateral cheeks, trunk and limbs. The lesions were asymptomatic and enlarged slowly. He was treated with intralesional betamethasone, oral thalidomide, Tripterygium glycosides and isotretinoin successively over several months, which showed no effect, and new lesions continued to develop.Upon dermatological examination, multiple dark-brown erythematous nodules and plaques were found on the cheeks, left eyebrow, right upper arm, abdomen, back, waist and right thigh. The largest plaque was on the right cheek (6 cm 9 8 cm) (Fig. 1a). No lymphadenopathy was found. Skin biopsies from lesions on the back and face revealed dense dermal and subcutaneous infiltrates composed essentially of histiocytes and lymphocytes (Fig. 1c). Immunohistochemistry showed that the histiocytes were positive for S-100 ( Fig. 1d) and CD68 (Fig. 1e), negative for CD1a (Fig. 1f), and some displayed emperipolesis.Cutaneous RDD was diagnosed. The patient was treated with combination therapy of rifampicin, ethambutol, itraconazole and thalidomide for 3 weeks but the lesions showed no response. Addition of methylprednisolone 40 mg/day for 3 days to the combination therapy also failed to bring any improvement.After reviewing the published work for refractory RDD, the patient was put on vincristine treatment. The initial dose of vincristine was 1 mg once a week. Efficacy was seen after three treatments and all lesions cleared after 12 treatments. The dose was then tapered to 1 mg every 2 weeks for 4 weeks and 1 mg every month for 4 months. No recurrence was found over a 6-month follow-up period (Fig. 1b).Rosai-Dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, was first described by Rosai and Dorfman 1 in 1969. Skin is most frequently involved in RDD, 2 and cutaneous RDD refers to those who only have skin lesions with no lymph node involvement. Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder with unknown etiology. Treatment is not essential for some patients because of the benign course of the disease, while appropriate therapy is necessary in patients with multiple/widespread lesions or in patients with rapid progression. Although various therapeutic strategies have been attempted and achieved successful results, such as surgery, radiation, cryotherapy, corticosteroids, thalidomide and dapsone, there are still no standard or satisfactory treatment options.In addition, chemotherapy can be considered for some severe or refractory patients. CVP (cyclophosphamide, prednisone and vincristine), and RCVP (rituximab, cyclophosphamide, vincristine and prednisolone) regimens have shown good response in some patients. 3,4 In our case, after failure of various therapies that had been reported effective for RDD, we tried vincristine, to which the patient responded very ...

Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease

“…84 A patient with multiplyrelapsed nodal RDD responded to cytarabine/prednisone/ vincristine followed by MTX/6-MP maintenance. 85 Successful treatment of refractory cutaneous RDD was reported with singleagent vincristine 86 and low-dose MTX. 87 In addition, azathioprine and interferon-a induced long-term remissions in patients with RDD.…”

Enfermedad de Rosai-Dorfman asociada a Miastenia Gravis

“…La cirugía puede ser curativa en casos unifocales, pero tiene utilidad limitada ante múltiples lesiones recurrentes (14). Algunos reportes indican beneficio con metotrexato oral en dosis bajas (15) o con vincristina como agente único (16). Otros medicamentos inmunomoduladores como talidomida (17), lenalidomida (18) o interferón (19) también han mostrado actividad; sin embargo, su perfil de toxicidad debe considerarse previo al inicio del tratamiento.…”

Successful treatment of disseminated Rosai-Dorfman disease with siltuximab

“…IgG4-related diseases have been considered an etiology when IgG4 + plasma cells were described in RDD lesions along with elevated serum IgG4 levels, but these have not been consistent. 5 Mutations in MAPK/ERK pathway have been document-ed in 33% of RDD patients in a retrospective study, and interestingly, the mutational profile was correlated with the location of disease -head and neck-, but not with clinical outcomes. 6 The diagnosis of RDD is heavily reliant on histology and immunohistochemistry.…”

“…1,10,11 Extensive and systemic disease, as in this case, have been managed with a variety of largely ineffective therapies including steroids, rituximab, and cytotoxic chemotherapy with vinca alkaloids, anthracyclines, alkylating agents and antimetabolites. 2,5,9,12,13 Details of these are limited to small studies and case reports, and nothing has been shown to be of consistent long-term benefit. Most recently, a case of RDD with activating KRAS mutation was treated with targeted therapy of cobimetinib, but again with limited follow up of a radiographic response after two months.…”