Cutaneous Rosai-Dorfman disease.
Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
Khoo
Joon Joon
JJ
Department of Pathology, Sultanah Aminah Hospital, Johor, Malaysia. khoo.joon.joon@med.monash.edu.my
Rahmat
Bin Othman
BO
D000328:Adult; D018949:Antigens, CD1 / Q000096:biosynthesis; D005260:Female; D015618:Histiocytosis, Sinus / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D009418:S100 Proteins / Q000096:biosynthesis; D012871:Skin Diseases / Q000378:metabolism / Q000473:pathology*
D018949:Antigens, CD1; C419393:CD1a antigen; D009418:S100 Proteins