Cutaneous Rosai- Dorfman Disease with Associated Uveitis

56 PRACTICAL DERMATOLOGY AUGUST 2014 R osai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML) is a benign lympho-histiocytic proliferative disorder initially described with bilateral painless lymphadenopathy (90 percent), fever, leukocytosis, elevated ESR, anemia, and polyclonal hypergammaglobulinemia (90 percent).1 RDD presents with cervical adenopathy most commonly in children or young adults (median age, 20 years), in those of African ancestry and male sex.1 Extranodal disease occurs in 25-40 percent, is often widespread, involving skin, respiratory tract, soft tissue, paranasal sinuses, visceral organs, bone, central nervous system, genitourinary tract, and orbit.2,3 Cutaneous RDD (CRDD) is rare with about 3/100 cases of SHML. CRDD has a median age 44 with a female and Asian predominance, and can be in any location. CRDD presents as red-brown-yellow localized or disseminated papules, plaques, or nodules.2-4