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Surgical Management of Cutaneous Rosai Dorfman Disease on the Scalp, a Rare Presentation and Review.
Anthony DeLeonibus MD, Morgan Fish MD, Christina Dami Lee DO, Raffi Gurunian MD PhD
Cleveland Clinic Foundation
2021-02-15
Presenter: Anthony DeLeonibus
Affidavit:
The material proposed for presentation in this abstract has not been published in any scientific journal or previously presented at a major meeting.
Director Name: Steven Bernard
Author Category: Resident Plastic Surgery
Presentation Category: Clinical
Abstract Category: General Reconstruction
Rosai Dorfman Disease (RDD) is a rare hematological disease that most notably presents in the lymphoid tissue as focal or diffuse lymphadenopathy. Cutaneous Rosai Dorfman Disease (CRDD) is less common and presents with skin findings in the absence of nodal involvement. In rare occurrences when CRDD presents in the subcutaneous tissue it can become invasive destroying neighboring structures.
In this unique presentation of the 5th recorded instance of CRDD in the scalp, complex scalp reconstruction was employed to reconstruct the anterior scalp. In a review of the RDD literature, surgical endeavors are routinely adjuvant to medical management but surgery has become a mainstay in treatment when CRDD tumor burden is large, invasive, or involving cosmetically sensitive areas.
This case involved a 60-year-old female with a slowly enlarging biopsy-proven CRDD mass on her forehead. Due to the aesthetic concerns based on the location of the mass, surgical excision of the approximately 9 x 4 cm mass including the skin, subcutaneous tissue, galea, and pericranium was performed. Reconstruction was successfully achieved with burring the calvarial outer table and scalp rotational flap measuring 10 x 16 cm based on the superficial temporal vessels. At 1 month follow up, her deep surgical margins were negative without any further plans for treatment.
CRDD is a unique condition that is overlooked due to its rare incidence and misdiagnosed without biopsy and immunohistochemistry analysis. In a review of the literature, extranodal CRDD management with steroids, chemotherapy, and radiation is not well-established and surgical intervention is often necessary.
