Korean J Dermatol.  2013 Oct;51(10):809-813.

Cutaneous Rosai-Dorfman Disease (RDD) Mimicking Primary Cutaneous Marginal Zone B-cell Lymphoma

• Noh TK
• Won KH
• Rhee DY
• Won CH
• Chang SE
• Lee MW
• Choi JH
• Moon KC

Abstract

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is an idiopathic, benign, histiocytic proliferative disease involving lymph nodes and extranodal sites. A 52-year-old woman presented with a 2-year history of multiple non-tender, erythematous nodules on her face. The initial punch biopsy showed lymphoid follicle-like aggregation of CD20 positive lymphocytes in the papillary dermis consisting of primary cutaneous marginal zone B-cell lymphoma. She underwent a 3-month course of radiotherapy, but the lesions progressed. The re-biopsy specimen revealed marked histiocytes with positive CD68 and S-100 proteins. The patient was treated with prednisolone and isotretinoin to which she responded slightly. Repeated histopathologic examinations enable us to make a definite diagnosis. Differentiating these two disorders as early as possible is crucial for appropriate treatment.

Keyword

Rosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy

MeSH Terms

Biopsy
Dermis
Diagnosis
Female
Histiocytes
Histiocytosis, Sinus*
Humans
Isotretinoin
Lymph Nodes
Lymphocytes
Lymphoma, B-Cell, Marginal Zone*
Middle Aged
Prednisolone
Radiotherapy
S100 Proteins
Isotretinoin
Prednisolone
S100 Proteins