Destombes-Rosai-Dorfman (DRD) disease with nasal and lymph-node involvement.

INTRODUCTION

Destombes-Rosai-Dorfman (DRD) disease was first described in 1965 by the pathologist Paul Destombes. Almost 90% of patients show lymph-node involvement, which is usually cervical, although all organs can be affected.

CASE REPORT: A 74-year-old woman consulted for left nasal obstruction and bilateral axillary lymphadenopathies. Clinical examination found a mass in the anterior left nasal cavity and several bilateral axillary lymphadenopathies, which were neither painful nor inflammatory. General health status was normal. Nasal and nodal samples showed large histiocytes with clear cytoplasm and emperipolesis. Within the histiocytes, immunohistochemistry found intense diffuse PS100 and CD68 expression and absence of CD1a marking. Progression was spontaneously favorable, without treatment.

DISCUSSION: DRD disease is a benign non-Langerhans histiocytosis, with many and varied clinical presentations. The pathophysiology is not known. Evolution is usually spontaneously favorable, but treatment is required in case of compression, obstruction or lysis, especially in bone, ENT or neurologic involvement.