Cutaneous rosai-dorfman disease and morphea: coincidence or association?
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.
Chappell
Jeaneen A
JA
Department of Dermatology, Saint Louis University School of Medicine, St. Louis, Missouri 63104, USA.
Burkemper
Nicole M
NM
Frater
John L
JL
Hurley
M Yadira
MY
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003324:Coronary Artery Disease / Q000150:complications; D003924:Diabetes Mellitus, Type 2; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D006973:Hypertension / Q000150:complications; D007150:Immunohistochemistry; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D014221:Triamcinolone / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D014221:Triamcinolone