Rosai-Dorfman Disease: A Case Report With Nodal and Cutaneous Involvement and Review of the Literature.
: Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes with an unknown etiology. It is a rare disease characterized by overproduction and accumulation of histiocytes within lymph node sinuses and many other extranodal sites, including skin, oral and nasal cavities, respiratory tract, eyelid, and periorbital area. In this case, a 44-year-old woman with diagnosis of Rosai-Dorfman disease, with xanthelasma-like cutaneous lesions on facial area, extending to her neck and acneiform papules on her back, cervical lymph node involvement, and concomitant presence of diabetes insipidus was presented. Histopathological examination of the lesions demonstrated diffuse lymphocyte, plasmocyte, eosinophil, and sparse neutrophil infiltration, together with histiocytes showing phagocytosed inflammatory cells (emperipolesis). Histiocytes demonstrated immunoreactivity with the antibodies for CD68 and S100, whereas they were negative for CD1a and Langerin. Laboratory tests were normal, except mild immunoglobulin G hypergammaglobulinemia. Systemic methylprednisolone therapy was effective for cutaneous lesions.
Available from: Julia Upton
- "High IgG has been noted in some benign hematological conditions such as the histiocytic disease Rosai– Dorfman (Kutlubay et al. 2014) and idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia (Kurosawa et al. 2009) and Castleman's disease (Hanada et al. 2012). Given that the underlying genetic etiology of these conditions is not understood and there is profound immune dysregulation, they have been included in this review as conditions possibly reflective of a primary immunodeficiency. "
09/2014; 2(2):150113084544003. DOI:10.14785/lpsn-2014-0019
Journal of the European Academy of Dermatology and Venereology 01/2015; DOI:10.1111/jdv.12967 · 2.83 Impact Factor
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ABSTRACT: Adult xanthogranuloma presents most commonly as an orange-tan firm solitary nodule with no systemic manifestations. Recently, some cases have been reported in conjunction with lymphoproliferative disorders. Adult reticulohistiocytosis classically presents as red to yellow-red dermal nodules. In the multicentric form, lesions have a predilection for hands and elbows, with a classic coral bead periungual presentation, and are often associated with symmetric erosive arthritis, particularly of the hands and wrists. The presentation and course of Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, can vary. The classic presentation is extensive, painless bilateral cervical lymphadenopathy, but some cases have been entirely extranodal.
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Dermatologic clinics 07/2015; 33(3):465-473. DOI:10.1016/j.det.2015.03.011 · 1.69 Impact Factor
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