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Cutaneous Rosai-Dorfman disease.
Melissa A Rubenstein1, Neil N Farnsworth, Josie A Pielop
1Department of Dermatology, Baylor College of Medicine, USA.
Dermatology Online Journal
|April 28, 2006
Summary
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) can rarely present as purely skin nodules. Differentiating this rare cutaneous form requires a high index of suspicion due to non-specific symptoms.
Area of Science:
- Dermatology
- Histopathology
- Oncology
Background:
- Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare, benign histiocytic proliferative disorder.
- While typically involving lymph nodes, RDD can uncommonly manifest as a purely cutaneous form without systemic involvement.
Observation:
- This study reports three cases of patients presenting with asymptomatic, enlarging skin nodules.
- These patients showed no evidence of lymphadenopathy or internal organ disease.
Findings:
- Histopathologic examination revealed characteristic features of RDD in skin lesions, including proliferating histiocytes with phagocytosed inflammatory cells.
- Differential diagnoses included dermatofibroma, spindle cell neoplasms, infectious granulomas, and xanthohistiocytic proliferations, highlighting diagnostic challenges.
- One patient responded to oral steroids, and another experienced spontaneous regression.
Implications:
- Diagnosing purely cutaneous RDD is challenging due to its rarity, non-specific clinical presentation, and overlapping histopathological features.
- A high index of suspicion among clinicians and pathologists is crucial for accurate diagnosis and appropriate management of this rare condition.