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Rosai-Dorfman Disease Case Report.

Naif M Altamimi¹, Vivian Li², Hershel Kahn³

¹Department of Dermatology, University of Hail, Hail, Saudi Arabia.

Case Reports in Dermatology

|July 17, 2025

Summary

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder. This case highlights a unique presentation of cutaneous RDD with dual morphologies, emphasizing the need for high diagnostic suspicion.

Keywords:

Dome-like lesions Histiocytic diseases Rosai-Dorfman disease Sinus histiocytosis with massive lymphadenopathy

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Area of Science:

Dermatology
Histopathology
Oncology

Background:

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder characterized by histiocyte accumulation, typically in lymph nodes.
Cutaneous RDD is an uncommon manifestation, representing less than 10% of all RDD cases.

Observation:

An 18-year-old female presented with a unique combination of a dome-like lesion overlying an indurated plaque.
Biopsies confirmed Rosai-Dorfman disease, prompting further oncologic evaluation.

Findings:

Positron emission tomography revealed hypermetabolic subcutaneous uptake in the thighs and lumbar regions.
Increased tracer uptake was also noted in the thoracic aorta and atria, suggesting systemic involvement.

Implications:

This case underscores the importance of considering RDD in dermatological diagnoses, especially with unusual presentations.
The presence of dual morphologies necessitates a high index of suspicion to differentiate from single pathological processes.