Volume : IV, Issue : I, January - 2015

CUTANEOUS ROSAI DORFMAN DISEASE

Dr. Bezwada Srinivasa Rao

Abstract :

Rosai-Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a benign disorder of histiocyte proliferation that usually affects the lymph nodes. RDD most frequently seen in children and young adults. The disease is more common in males and has 2:1 male -to-female ratio. The most frequent clinical presentation of RDD is a massive bilateral and painless cervical lymphadenopathy with fever, night sweats, weight loss, leucocytosis, an increased erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia. More than 40% of patients affected by RDD have extranodal involvement. Skin involvement is one of the most common extra nodal manifestation apart from involving nasal cavity, eye, thyroid, pancreas or in other extra nodal sites. Cutaneous Rosai-Dorfman disease (C-RDD) is a distinct entity with unknown etiology, oadly different from systemic RDD, confined to the skin without lymphadenopathy. Primary CRDD without lymphadenopathy is rare. We present a rare case of extra nodal RDD in a 32 yr old female patient presented with multiple tender erythematous indurated subcutaneous plaques over abdominal wall, buttocks, lower limbs with left periorbital swelling. Histologically scaterred and clusters of large histiocytes intermingled with mixed inflammatory cells along with emperipolesis or lymphophagocytosis, the hallmark feature that is diagnostic of RDD is seen.

Keywords :

Article: Download PDF   DOI : 10.36106/ijsr  

Cite This Article:

Dr.Bezwada Srinivasa Rao Case Report Cutaneous Rosai Dorfman Disease International Journal of Scientific Research, Vol : 4, Issue : 1 January 2015

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