Cutaneous Rosai-Dorfman disease

Area of Science:

• Dermatology
• Pathology
• Histiocytosis

Background:

• Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder.
• Cutaneous Rosai-Dorfman disease (cRDD), without lymph node involvement, is exceptionally rare.
• Diagnosis can be challenging due to non-specific clinical presentations.

Observation:

• A case of cRDD in a 34-year-old female presenting with a chest nodule and satellite lesions is described.
• Histopathological examination revealed characteristic large histiocytes (Rosai-Dorfman cells) with emperipolesis.
• Immunohistochemistry confirmed S-100 protein positivity and CD 1a negativity.

Findings:

• The patient had no lymphadenopathy or extranodal lesions.
• Serological tests indicated prior Epstein-Barr virus and cytomegalovirus infections.
• Microscopic findings were crucial for differentiating cRDD from other skin conditions.

Implications:

• This case underscores the importance of a high index of suspicion for diagnosing cRDD.
• Histopathological confirmation is essential, especially when lymphadenopathy is absent.
• Clinicians and pathologists must be aware of this rare entity to ensure timely diagnosis and management.