Cutaneous Rosai-Dorfman disease: A separate clinical entity

Welt

et al. 2018

ARS Medica Tomitana

Rosai-Dorfman disease (RDD) is a rare idiopathic benign disease, self-limited non-Langerhans cell histiocytosis, most frequently presented as a massive bilateral and painless lymphadenopathy, associated with fever and weight loss. Extranodal manifestations of RDD represents a true diagnosis challenge for clinicians and surgical pathologists. A 64-year-old female, known with arterial hypertension and type 2 diabetes, presented to our Surgery Clinic for a left arm painless cutaneous tumour, having its onset one year before, and rapidly enlarged in the last two months. Physical examination revealed a firm and elastic cutaneous nodular lesion of 4 x 2 cm in size, prominent to the skin, with central ulceration area of 10 x 5 mm, located on the posterior side of the left arm. Surgery was performed, with lymphoma as a differential diagnosis at intraoperative extemporaneous examination. The RDD disease diagnosis was considered at histopathological analysis and confirmed by immunohistochemistry. Herein, we describe a rare case of purely cutaneous RDD presenting as unique cutaneous ulcerative nodular lesion, surgical treated only, and without local relapse after one-year follow-up.

Section: Discussionmentioning

confidence: 99%

Purely Cutaneous Rosai-Dorfman Disease: a True Clinical Diagnosis Challenge

Welt

et al. 2018

ARS Medica Tomitana

“…Rosai-Dorfman Disease (RDD) is a benign disorder of proliferative histiocytosis, that most often presents with lymphadenopathy. [1][2][3][4][5] RDD typically affects cervical lymph nodes and is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR), and polyclonal hypergammaglobulinemia. [1][2]5 Extranodal involvement may occur, with the skin being the most common site.…”

Section: Introduction Case Reportmentioning

confidence: 99%

“…In the latter scenario, the disease is classified as Cutaneous Rosai-Dorfman Disease (CRDD). [1][2][3][4][5] CRDD is believed to be a distinct entity because of its unique epidemiologic pattern and the absence of systemic manifestations. [4][5] It is also considered a rare entity, with only a few cases reported in the literature.…”

mentioning

confidence: 99%

“…[1][2][3][4][5] CRDD is believed to be a distinct entity because of its unique epidemiologic pattern and the absence of systemic manifestations. [4][5] It is also considered a rare entity, with only a few cases reported in the literature. 5 We report the case of a biopsy and immunohistochemistry proven CRDD, in a young African American woman.…”

mentioning

confidence: 99%

“…[4][5] It is also considered a rare entity, with only a few cases reported in the literature. 5 We report the case of a biopsy and immunohistochemistry proven CRDD, in a young African American woman.…”

mentioning

confidence: 99%

See 2 more Smart Citations

Cutaneous Rosai-Dorfman Disease: A Case Report

Shihab¹,

Talbott²,

Kahn³

et al. 2018

J of Skin

Cutaneous Rosai-Dorfman Disease (CRDD) is an uncommon benign histiocytosis of unknown etiology. CRDD is oftentimes misdiagnosed because it has variable clinical manifestations, particularly in the absence of lymphadenopathy. We report a case of a 23-year-old African American woman presenting with clustered nodular plaques on her right thigh, buttocks, back, and chest for the past two years. History, clinical, histopathological, and immunochemistry findings corresponded with CRDD, and as such, she was treated with halobetasol propionate 0.05% cream twice daily.

Ann of Pathol and Lab Med

Cutaneous Rosai-Dorfman Disease – A Case Report and Review of Literature

Thomas¹,

Joseph²,

Napolean³

et al. 2022

Rosai–Dorfman disease (RDD), or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a self-limiting, rare benign proliferative disorder of histiocytes in the lymph nodes with occasional extra-nodal involvement of the skin. Isolated Cutaneous Rosai-Dorfman disease(C-RDD) without node involvement is an exceedingly rare occurrence. Despite its unique characteristics, the diagnosis of Cutaneous Rosai Dorfman disease is hampered by its variable clinical presentation, misleading histopathological patterns, and the absence of lymphadenopathy. Herein we present a case report of Cutaneous Rosai-Dorfman disease without any lymph node involvement.