Cutaneous Rosai-Dorfman disease

Area of Science:

• Dermatology
• Pathology
• Oncology

Background:

• Rosai-Dorfman disease (RDD) is a rare, benign histiocytic proliferative disorder.
• It is typically characterized by sinus histiocytosis with massive lymphadenopathy.
• Extranodal and purely cutaneous forms of RDD are exceptionally rare.

Observation:

• A 63-year-old woman presented with multiple nodules at a recent biopsy site.
• Initial examination and CT scans revealed no clinical lymphadenopathy.
• A subsequent skin biopsy confirmed changes consistent with Rosai-Dorfman disease.

Findings:

• The patient's presentation represented a rare extranodal, purely cutaneous manifestation of Rosai-Dorfman disease.
• The nodules developed at the site of a previously biopsied histiocyte proliferation.
• CD 68+ histiocytes were identified in the initial biopsy.

Implications:

• Early diagnosis and appropriate management are crucial for cutaneous Rosai-Dorfman disease.
• While spontaneous regression can occur, surgical intervention is the most effective treatment.
• Systemic therapies like high-dose thalidomide show promise for extensive cutaneous involvement.