FDG PET Scan in Cutaneous Rosai-Dorfman-Destombes Disease

Abstract

Rosai-Dorfman-Destombes (RDD) disease is also called as sinus histiocytosis and is characterized by enlarged lymph nodes and previously called as non-Langerhans cell histiocytosis. Based on pathologic, molecular, and genetic features, RDD disease has been classified into sporadic noncutaneous (classical nodal, extranodal, neoplasia associated, and autoimmune associated), familial (H syndrome, autoimmune lymphoproliferative syndrome related, and familial NOS), and cutaneous subtypes. Cutaneous RDD disease is not associated with lymphadenopathy or visceral organ involvement. The disease is usually localized and has relatively better long-term prognosis. Presented here is a case of indurated plaque-like skin lesions over the abdomen. ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography scan revealed FDG avid cutaneous-subcutaneous soft-tissue lesions. Histology confirmed the diagnosis of cutaneous RDD disease.

Keywords: Cutaneous Rosai–Dorfman–Destombes disease; Rosai–Dorfman–Destombes; fluorodeoxyglucose positron emission tomography scan.

Figure 1

Clinical picture shows nodular - Hyperpigmented lesion over the lateral thoracoabdominal wall with irregular margins (a). Clinically considered to be either Hansen’s disease or Actinomycosis. Skin biopsy showed dense and diffuse infiltrates of lymphocytes and numerous plasma cells and large foamy histiocytes. Infiltrate was spanning superficial and deep layers of the dermis. Foamy histiocytes in the center show emperipolesis (i). ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography computed tomography showed FDG avid three foci of radiotracer uptake along the right side of the body (f). There corresponded to the cutaneous thickening seen clinically (b and c), An additional subcutaneous lesion was seen in the right high gluteal region (g and h). FDG avid right axillary (j and k) and left level II neck nodes were also noted (d and e)