Cutaneous Rosai-Dorfman disease: a systematic review and reappraisal of its treatment and prognosis.

Cutaneous Rosai Dorfman disease (CRDD) is a rare histiocytic disorder that shows distinctive clinical presentation and prognosis. Sufficient data is currently lacking regarding evidence-based management of CRDD. This systematic review aims to provide a comprehensive overview of CRDD, focusing on treatment approaches and outcomes. PubMed and Scopus databases were searched for studies on CRDD from June 1st, 2013 to May 31st, 2023. Articles describing cases of CRDD confirmed with histological examination were eligible for inclusion. All interventions for CRDD were analyzed. The primary outcome measure was the response of cutaneous lesions to treatment including complete response (CR), partial response (PR), and no response. The secondary outcome measures were mortality rate, relapse rate, and the occurrence of adverse events related to CRDD treatment. Eighty-seven articles describing 118 CRDD cases were included. The mean age was 48.2±16.8 years. The sex ratio (F/M) was 1.53. Nodular (46.6%) erythematous (45.3%) lesions, located on the face (38.1%) were the most prevalent presentations. Associated hematological malignancies were noted in 8 (6.8%) cases. Surgical excision was the most prevalent intervention (51 cases) with CR in 48 cases. Systemic corticosteroids were used in 32 cases with 20 CR/PR, retinoids in 10 cases with 4 CR/PR, thalidomide in 9 cases with 5 CR/PR, methotrexate in 8 cases with 7 CR/PR while observation was decided in 10 cases with 6 CR/PR. Factors independently associated with the absence of response to treatment were facial involvement (OR = 0.76, p = 0.014), and cutaneous lesion size (OR = 1.016, p = 0.03). This systematic review shows distinctive clinical characteristics of CRDD and provides insights into the appropriate management of the disease. It allowed a proposal of a treatment algorithm that should be interpreted in the context of current evidence and would help practitioners in treating this rare disease.

A rare case of facial cutaneous Rosai-Dorfman disease clinically mimicking basal cell carcinoma followed by multiple myeloma after 2 years.

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis disorder characterized by the proliferation of histiocytes within the lymph nodes. Extranodal involvement can occur; however, only 10% of extranodal RDD involve the skin. We present a unique case of a 66-year-old woman with cutaneous RDD followed by the development of multiple myeloma (MM). To our knowledge, this is only the second reported case where RDD preceded a diagnosis of MM, with the first documented instance occurring in 2018. The patient presented to the dermatology clinic with a 5-year history of painless, solitary lesion over the right cheek. Local examination revealed a single 6 mm x 7 mm well-circumscribed pearly telangiectatic lesion resembling basal cell carcinoma over the right nasolabial fold and cheek. The lesion was excised with a 3 mm circumferential margin. Histopathology showed a mixed lymphohistiocytic cell infiltrate with emperipolesis and immunohistochemical staining patterns consistent with RDD. Two years later, the patient presented with hip pain and was diagnosed with MM. She was treated with lenalidomide, bortezomib, and dexamethasone, and was later maintained on lenalidomide. Our case adds to the limited evidence suggesting a potential association between RDD and MM. Further research in this field is required to promptly identify and manage patients with such a presentation in the future.

Enfermedad de Rosai-Dorfman cutánea, reporte de un caso / Cutaneous Rosai-Dorfman disease, a case report

RESUMEN La enfermedad de Rosai-Dorfman cutánea (ERDC) es una rara proliferación reactiva de histiocitos, que cursa con lesiones cutáneas sin compromiso generalizado. Las manifestaciones clínicas cutáneas son diversas y la variante clínica "tipo tumoración" es la forma más infrecuente. Se describe el caso de un paciente con ERDC que se presentó con esta forma inusual.

ABSTRACT Cutaneous Rosai-Dorfman disease (CRDD) is a rare reactive histiocytic proliferation, which presents with skin lesions without systemic involvement. The clinical cutaneous manifestations are diverse and the "tumor-like" clinical variant is the most infrequent. We describe the case of a patient with CRDD who presented with this unusual form.

Dermoscopy combined with reflectance confocal microscopy as a noninvasive involvement for monitoring Cutaneous Rosai-Dorfman: A case report.

Cutaneous Rosai-Dorfman disease(CRDD) is an extremely rare entity and features histiocytic proliferation in the skin. Dermoscopy and reflectance confocal microscopy(RCM) reports on CRDD are rare. We reported a case of CRDD and summarized the dermoscopy(FotoFinder Medicare 800HD, FotoFinder-Systems GmbH, Birbach Germany) and RCM(VivaScope® 1500, V Caliber Imaging and Diagnostics) features of CRDD. The dermoscopic features of CRDD showed red-orange background with pale yellowish roundish areas similar to millet, surrounded by branched blood vessels. Sometimes the white structureless materials of CRDD could be observed by dermoscopy, which may be a hint of spontaneous regression. The RCM features of CRDD revealed dense highly refractile roundish or ovoid structures(inflammatory cells), and multiple larger structures with central low refraction and moderately refractive peripheral semicircle or circle(engulfed inflammatory cells), together with low refractive branched structures(blood vessels). Dermoscopic and RCM features of CRDD can help the dermatologists recognize and follow-up the disease in real time.

Fractional laser combined with 5-Aminolevulinic acid photodynamic therapy for the treatment of Cutaneous Rosai-Dorfman Disease: A case report.

Rosai-Dorfman disease(RDD) is a rare benign histiocytic proliferative disorder. Cutaneous Rosai-Dorfman disease(CRDD)is a rare variant of RDD with lesions localized to the skin. We report a case of patients with CRDD who was refractory to methotrexate, thalidomide, cyclosporine, glucocorticoid for local injection and external use, but resolved with fractional laser combined with 5-aminolevulinic acid photodynamic therapy (ALA-PDT). We believe that fractional laser technology combined with ALA-PDT for CRDD is a minimally invasive, effective and satisfactory treatment.

Pure Cutaneous Rosai Dorfman Disease: An Uncommon Location for a Rare Histiocytic Proliferative Disorder.

RosaiDorfman disease (RDD) is a rare, self-limiting disease of uncertain etiology involving lymph nodes as well as extranodal sites. Isolated or pure cutaneous RDD (PCRDD) without lymph node involvement is very unusual accounting for only 3% of all described cases of RDD. The clinical features of PCRDD are quite different from RDD according to the literature on extranodal RDD, thereby emphasizing that PCRDD is a distinct clinical entity. Histopathology remains the gold standard for the diagnosis of both systemic and PCRDD with the presence of characteristic emperipolesis observed in histiocytes while immunohistochemistry (IHC) (S100, CD 68 positive, and CD 1a-negative) serves as a useful adjunct. We hereby report a case of a 36-year-old female who presented with a tender, indurated plaque on the left forearm with a clinical differential diagnosis of borderline tuberculoid leprosy/sarcoidosis and lupus vulgaris. However, on histopathology, a diagnosis of RDD was performed and confirmed on IHC. This case highlights the need to create awareness among young pathologists and clinicians about PCRDD to prevent overzealous treatment.

5-Aminolevulinic acid photodynamic therapy combined with CO2 laser therapy for the treatment of cutaneous Rosai-Dorfman disease: A case report.

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. CRDD is a rare form of Rosai-Dorfman disease that has various clinical presentations and multiple possible therapies with varying efficacies. ALA-PDT combined with CO2 laser therapy was safe and effective for treating CRDD in the current patient, with no recurrence during the 3 months of follow-up medical examinations. This case suggests that ALA-PDT combined with CO2 laser can be used to treat patients with CRDD.

Emergence of cutaneous Rosai-Dorfman disease during immunosuppressive treatment of follicular B-cell lymphoma: A case report.

BACKGROUND: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare proliferation of non-Langerhans histiocytes. Cutaneous Rosai-Dorfman disease is a rare subtype of Rosai-Dorfman disease limited to the skin with variable clinical presentation. CASE SUMMARY: A 59-year-old female with a history of osteoarthritis, hypothyroidism, and follicular B-cell lymphoma presented with pruritic, erythematous, dome-shaped papules that developed while on chemotherapy treatment. During cutaneous disease progression, the patient was further diagnosed with myelodysplastic syndrome. Histology review revealed patchy staining for S100 in the CD68+ histiocytes within the dermis with no enlarged histiocytes or emperipolesis. Given the absence of other findings, this was interpreted as cutaneous Rosai-Dorfman disease. CONCLUSION: There is still little known about the aetiology and pathogenesis of cutaneous Rosai-Dorfman disease. Non-specific immunohistochemistry in the midst of lymphoma, immunosuppressive treatments, and myelodysplastic syndrome produced a blurred diagnostic picture and delayed appropriate treatment, highlighting the diagnostic challenges of cutaneous Rosai-Dorfman disease.

Cutaneous Rosai-Dorfman Disease: A Treatment Challenge.

Rosai-Dorfman disease (RDD) is a benign histiocytosis that rarely manifests as a purely cutaneous form. Its differential diagnosis and treatment can pose a challenge to both clinicians and pathomorphologists. We present the case of a 69-year-old woman with cutaneous RDD presenting as multiple nodules on the right thigh who was treated with low-dose methotrexate weekly for > 1 year with a partial response.

Self-Limited Primary Cutaneous Rosai-Dorfman Disease: A Case Report and Literature Review.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, benign, and self-limiting disease. Cutaneous Rosai-Dorfman disease (CRDD) is an exceedingly rare extranodal variant, which is strictly limited to the skin and is easily misdiagnosed. We present the case of a 47-year-old Chinese woman with primary CRDD and a review of relevant literature. The patient had red-yellow papules on her left cheek and chest for more than three months with occasional tenderness. Histopathology showed phagocytic histiocytes in the dermis with the typical presentation of emperipolesis. Immunohistochemistry (IHC) staining showed S100 (+), CD68 (+), and CD1a (-). Based on these findings and the negative systemic presentation, the patient was diagnosed with primary papulonodular-type CRDD, and no treatment regimen was initiated. After three months, the skin lesions partially disappeared, confirming the spontaneous regression of CRDD.

Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report

Introduction@#Rosai-Dorfman disease is a rare disease that manifests with painless cervical lymphadenopathy, fever, anemia, an elevated erythrocyte sedimentation rate (ESR), and hypergammaglobulinemia. Extranodal lesions occur in 1/3 of patients, and the skin is involved in more than 10% of cases. Purely cutaneous disease is uncommon and only about more than 100 cases have been reported. Cutaneous Rosai-Dorfman Disease (CRDD) appears to be a distinct entity with different age and race predilection from cases with lymph node involvement.@*Case report@#This is a case of a 40-year-old Filipino female who presented with multiple erythematous papules and plaques with pustules on the cheeks. Skin punch biopsy showed a dense dermal infiltrate of polygonal histiocytes with abundant cytoplasm and vesicular nuclei. Emperipolesis was also present. The histiocytes were highlighted by the immunohistochemical stains S-100 and CD68 and was CD1a negative. Complete blood count and ESR were normal. Cervical lymphadenopathy was absent. Findings were consistent with Cutaneous Rosai-Dorfman disease. The patient was started on methotrexate at 15mg/week with folic acid supplementation. Mild soap, benzoyl peroxide 5% gel and tretinoin 0.05% cream once daily were maintained during the treatment course. There was significant decrease in erythema and size of existing lesions after 2 months. The patient was referred to a hematologist for monitoring of possible future systemic involvement.@*Conclusion@#Because of its rarity, clinicopathological correlation is always mandatory to establish a diagnosis of CRDD. Immu- nohistochemical stains are required to differentiate this entity form other forms of Langerhans cell histiocytosis. Multidisci- plinary referral is required to rule out concomitant systemic involvement.

Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant.

Primary cutaneous Rosai-Dorfman disease is a rare form of Rosai-Dorfman disease limited to the skin. The diagnosis of primary cutaneous disease is based on a combination of clinical presentation, histopathology, and the detection of S100+, CD68+, and CD1a- histiocytic immunophenotyping. However, the diagnosis of primary cutaneous disease is often difficult and significantly delayed due to the non-specific nature of its histologic and clinical features. In this review, we describe four cases in order to familiarize pathologists and dermatopathologists with the clinicopathologic correlation of primary cutaneous Rosai-Dorfman disease and to help facilitate early diagnosis. In addition, we discuss the proposed pathophysiology and molecular etiology of this tumor, and its relationship with IgG4 sclerosing disease.

Cutaneous Rasai-Dorfman disease / 中华皮肤科杂志

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder.The etiology and pathogenesis of RDD are still unclear,and may be associated with the activation of signal transduction pathways and inflammatory reactions induced by infection and immune dysfunction.CRDD is defined as the RDD involving only the skin.Histopathological examination of CRDD lesions shows that a large amount of histiocytes,lymphocytes and plasma cells infiltrate the dermis,and intact endocytosed lymphocytes can be observed in the cytoplasm of histiocytes,which is called emperipolesis.Immunohistochemical study shows that histiocytes are stained positive for S-100 and CD68,but negative for CD1a.The treatment of CRDD includes surgical excision,local therapy and systemic therapy.Lesions can subside spontaneously in some patients,and it usually has a favorable prognosis.