| Title | Cutaneous rosai-dorfman disease and morphea: coincidence or association? | | Author(s) | Chappell JA, Burkemper NM, Frater JL, Hurley MY | | Institution | Department of Dermatology, Saint Louis University School of Medicine, St. Louis, Missouri 63104, USA. | | Source | Am J Dermatopathol 2009 Jul; 31(5):487-9. | | Abstract | Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease. A third of patients with this disease have extranodal involvement affecting the skin. Of these individuals, only around 3% will have purely cutaneous Rosai-Dorfman disease, which is limited to skin manifestations without systemic involvement. Cutaneous (localized) scleroderma or morphea, on the other hand, is a more common disease that most often affects women of all ages. Both conditions have unknown etiologies. Presented here is a case of a 60-year-old white woman with cutaneous Rosai-Dorfman disease and coexisting morphea. Representative biopsies from both areas were performed: one showing a dermal S-100+ histiocytic infiltrate with emperipolesis and the other showing a deep perivascular and interstitial plasma cell infiltrate with dermal sclerosis and loss of perieccrine fat. A laboratory and radiologic workup revealed no evidence of systemic involvement by either entity. The diagnosis of coexisting cutaneous Rosai-Dorfman disease and morphea was established. To our knowledge, this is the first report of these 2 entities found simultaneously in 1 patient. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 19542928 |
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