Isolated Intraocular Rosai-Dorfman Disease.

Ocul Oncol Pathol. 2019 Oct;5(6):418-423

Authors: Fogt F, Rüediger T, Augustin AJ, Frank DM, Rosenwald A, Wellmann A, Lee V

Abstract
Background/Aims: To report a case of Rosai-Dorfman disease (RDD) presenting as a solitary, choroidal mass, initially suspicious for uveal melanoma, in a 72-year-old woman.
Methods: Retrospective case report of a single patient.
Results: A 72-year-old woman presented with sudden vision loss in the right eye. A month prior, visual acuity was 20/40, but she was noted to have a choroidal mass confirmed with B-scan ultrasonography. Patient’s vision deteriorated significantly a month later and a shallow retinal detachment was newly noted. Magnetic resonance imaging was obtained, demonstrating a hyperintense intraocular tumor on TI imaging. Patient underwent enucleation of the right eye for suspicion of a uveal melanoma. Pathology revealed a mixed cellular infiltrate with histiocytes, some exhibiting emperipolesis. Macrophage immunohistochemical stains were positive, while melanocytic markers were negative. A diagnosis of RDD was made. Subsequently, the patient had a negative workup for systemic involvement. A final diagnosis of intraocular RDD without extraocular and systemic involvement was determined.
Conclusion: We describe a rare presentation of RDD as a solitary choroidal mass in an elderly patient with overlapping features of uveal melanoma. Definitive diagnosis could only be made on histology. RDD should be considered in the differential diagnosis of a choroidal lesion in the elderly.

PMID: 31768365 [PubMed]

Rosai-Dorfman disease with cutaneous plaques and autoimmune haemolytic anemia.

BMJ Case Rep. 2019 Nov 24;12(11):

Authors: Subhadarshani S, Kumar T, Arava S, Gupta S

Abstract
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-Langerhan cell histiocytosis which primarily involves lymph nodes. Extranodal involvement in the form of cutaneous plaques can occur and can pose a diagnostic challenge because of pleomorphic presentation and histopathological mimics. Rarely, systemic autoimmune involvement may complicate the disease process. We present a 28-year-old woman with slowly evolving scaly erythematous cutaneous plaques and fluctuating lymphadenopathy, associated with autoimmune haemolytic anaemia. The patient responded favourably to oral corticosteroids and acitretin with significant flattening of cutaneous plaques, reduction in size of neck nodes and improvement of anaemia.

PMID: 31767610 [PubMed – in process]