Cutaneous Rosai–Dorfman disease

An intramedullary cervical cord lesion in a 12‐year‐old girl

“…Rosai–Dorfman disease (RDD), with characteristics of marked dilatation of the lymph node sinuses containing histiocytes, is a rare self‐limited pseudolymphomatous disease of unknown etiology. Rosai and Dorfman first described RDD as a massive proliferative lesion with systemic symptoms and lymphadenopathy in 1969 . The classic presentation for Rosai–Dorfman disease is painless bilateral cervical lymphadenopathy in a young asymptomatic patient, including nasal obstruction or hearing loss.…”

“…Rosai and Dorfman first described RDD as a massive proliferative lesion with systemic symptoms and lymphadenopathy in 1969. 1,2 The classic presentation for Rosai-Dorfman disease is painless bilateral cervical lymphadenopathy in a young asymptomatic patient, including nasal obstruction or hearing loss. Although lymph node involvement occurs most commonly, extranodal RDD has been reported in over 40% of cases and mostly occurs in the paranasal sinuses, skin, spine, skull base, orbit, testes and upper respiratory tract.…”

A 60-year-old woman with subcutaneous nodules on the thigh

“…Treatments for RDD include retinoids, thalidomide, antibiotics, cryotherapy, simple excision, topical and oral steroids. 5 The poorly documented benefit of these various approaches must be weighted carefully against their potential side-effects and the overall good prognosis of cutaneous RDD, which often subsides spontaneously. Our patient was treated with intralesional steroid injections for aesthetic reasons, which resulted in partial improvement.…”

Purely cutaneous rosai-dorfman disease with immunohistochemistry

“…Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign multi-organ non-Langerhans cell histiocytic proliferation that was first described in 1969 by Rosai and Dorfman. [ 1 2 ] The disease most commonly involves the lymph nodes but can be seen in virtually any part of the body; the presentation is usually painless massive bilateral cervical lymphadenopathy. Skin lesions occur in a minority of patients with systemic disease; nonetheless the cutaneous form of RDD, also known as cutaneous sinus histiocytosis, is a rarer entity and is detectable in only 3% of reported cases.…”