Destombes–Rosai–Dorfman disease of the anterior abdominal wall: Extranodal sinus histiocytosis with massive lymphadenopathy

Hubert Daisley Jr,Deandra Thomas-Romain,Martina Daisley,Sailaja Golamari,Lilly Paul,Dawn Meyers

doi:10.7241/ourd.20211.19

Abstract

estombes–Rosai–Dorfman disease—often simply referred to, in the literature, as Rosai–Dorfman disease (RDD)—is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. A subset of patients with RDD, however, display extranodal manifestations highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress when compared to the nodal manifestations. This study describes the case of a young African male presenting himself with multiple nodules involving the anterior abdominal wall, who was found to have extranodal RDD. The current mode of diagnosis and the clinical management of RDD are reviewed.

Highlights

Sinus histiocytosis with massive cervical lymphadenopathy is a non-neoplastic proliferative disease of histiocytes of unknown etiology
A few binucleated histiocytes resembled those of ReedSternberg cells (RS cells) were seen, there was a complete lack of expression of the markers CD15 (Fig. 2f) and CD30, which makes Hodgkin Lymphoma quite unlikely
The overall histological picture of the abdominal lesion was that described by Rosai and Dorfman in lymph nodes with sinus histiocytosis with massive lymphadenopathy

Summary

INTRODUCTION

Sinus histiocytosis with massive cervical lymphadenopathy is a non-neoplastic proliferative disease of histiocytes of unknown etiology. Destombes and Rosai-Dorfman disease of the anterior abdominal wall - Extra nodal sinus histiocytosis with massive lymphadenopathy. A few binucleated histiocytes resembled those of ReedSternberg cells (RS cells) were seen, there was a complete lack of expression of the markers CD15 (Fig. 2f) and CD30, which makes Hodgkin Lymphoma quite unlikely. The overall histological picture of the abdominal lesion was that described by Rosai and Dorfman in lymph nodes with sinus histiocytosis with massive lymphadenopathy. The histopathology of the skin lesion mimicked those seen in lymph nodes with sinus histiocytosis with massive lymphadenopathy. Emperipolesis was evident, which constituted intact lymphocytes and neutrophils in the cytoplasm Several binucleated histiocytes resembling Reed–Sternberg (RS) cells were seen, but there was a complete lack of expression of the markers CD15 and CD30, which made Hodgkin lymphoma quite unlikely. Immunohistochemical stains confirmed the diagnosis of extranodal RDD by showing positivity for CD68 and S100 (Figs. 2d and 2e)

DISCUSSION

CONCLUSION