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Cutaneous Rosai-Dorfman disease: a case report
Josie da Costa Eiras1, Antônio Pedro Mendes Schettini, Livia Lima de Lima
1Alfredo da Matta Foundation, Manaus, AM, Brazil.
Anais Brasileiros De Dermatologia
|December 15, 2010
Summary
Rosai-Dorfman disease (RDD), a rare non-Langerhans cell histiocytosis, can manifest purely in the skin. This case highlights the rarity of cutaneous RDD, emphasizing its potential for extranodal presentation.
Area of Science:
- Pathology
- Dermatology
- Oncology
Background:
- Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare benign histiocytic disorder of unknown cause.
- Typically presenting as cervical lymphadenopathy, RDD can also involve extranodal sites in up to 43% of cases.
Observation:
- This report details a rare instance of RDD exclusively affecting the skin.
- The case underscores the diverse clinical manifestations of RDD beyond nodal involvement.
Findings:
- Purely cutaneous Rosai-Dorfman disease represents an uncommon but recognized variant of the condition.
- Histopathological confirmation is crucial for diagnosing this rare extranodal presentation.
Implications:
- Recognizing purely cutaneous RDD is essential for accurate diagnosis and appropriate patient management.
- Further research into the etiology and specific treatments for extranodal RDD, including cutaneous forms, is warranted.
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