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Cutaneous Rosai-Dorfman disease
F J Child1, L C Fuller, J Salisbury
1Department of Dermatology, King's College Hospital, London, U.K.
Clinical and Experimental Dermatology
|July 17, 1998
Summary
This report details a rare case of purely cutaneous Rosai-Dorfman disease (RDD), a condition characterized by histiocytoid cells. The study highlights a solitary skin plaque presentation, emphasizing the rarity of this non-lymph node RDD manifestation.
Area of Science:
- Dermatology
- Pathology
- Immunology
Background:
- Rosai-Dorfman disease (RDD) typically involves lymph nodes, presenting as sinus histiocytosis.
- Cutaneous involvement occurs in a minority of RDD cases, often alongside lymphadenopathy.
- Purely cutaneous RDD, without lymph node involvement, is exceptionally rare.
Observation:
- A solitary, asymptomatic plaque was observed on the posterior left thigh of a patient.
- Histological examination revealed characteristic large histiocytoid cells with emperipolesis.
- This presentation represents one of only 18 previously reported cases of purely cutaneous RDD.
Findings:
- The patient's condition was diagnosed as purely cutaneous Rosai-Dorfman disease based on clinical and histological findings.
- The rarity of this presentation underscores the diverse clinical spectrum of RDD.
- The etiology remains unknown, but a reactive or immunological basis is suspected.
Implications:
- This case expands the known clinical presentations of Rosai-Dorfman disease.
- Further research into the pathogenesis of cutaneous RDD may elucidate its reactive or neoplastic nature.
- Understanding rare presentations is crucial for accurate diagnosis and management of RDD.