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Rosai-Dorfman syndrome.
Najeeba Riyaz1, Anza Khader, S Sarita
1Department of Dermatology, Medical College, Calicut, India. saif_gem@hotmail.com
Indian Journal of Dermatology, Venereology and Leprology
|January 6, 2006
Summary
Rosai-Dorfman syndrome, a rare condition, presents as sinus histiocytosis with massive lymphadenopathy. This case highlights a young woman with skin papules and enlarged lymph nodes, requiring oncologic management.
Area of Science:
- Dermatology
- Pathology
- Oncology
Background:
- Rosai-Dorfman syndrome is a rare, benign histiocytic proliferative disorder.
- It is characterized by sinus histiocytosis and massive lymphadenopathy.
- Cutaneous involvement is common but often presents with nodules rather than papules.
Observation:
- A 25-year-old female presented with multiple erythematous and yellowish papules on her face and thigh.
- She exhibited massive, generalized lymphadenopathy and mild hepatomegaly, with a normal hemogram.
Findings:
- Skin biopsy revealed histiocyte collections with emperipolesis and plasma cells.
- Lymph node biopsy demonstrated architectural distortion, dilated sinuses packed with histiocytes exhibiting lymphocytophagocytosis, anisonucleosis, and clear cytoplasm.
Implications:
- This case highlights the dermatological manifestations of Rosai-Dorfman syndrome.
- Accurate histopathological diagnosis is crucial for appropriate patient management and referral to oncology.