CASE REPORT
Facial asymmetry with nasal and orbital involvement in a case of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease)

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Summary

Sinus histiocytosis with massive lymphadenopathy or “Rosai–Dorfman”s syndrome is a rare benign disease of unknown etiology, usually seen in younger patients. A 12-year-old boy of the disease presented with facial asymmetry, massive lymphadenopathy, nasal and orbital manifestation is reported. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. Local excision of the orbital lesion and endonasal endoscopic resection of the disease from nasal, paranasal sinuses, and nasopharynx was achieved effectively. The clinical presentation, histologic characteristics, pathogenesis and treatment of this case is discussed. This is the first reported case, to the best of our knowledge, of the disease to be associated with facial asymmetry.

Introduction

Rosai–Dorfman disease, with its synonym of sinus histiocytosis with massive lymphadenopathy (SHML), is a distinct clinicopathological entity first described by Rosai and Dorfman [1]. This disease is a benign histiocytic proliferation frequently seen in younger patients. It is usually an indolent disease lasting several months but not endangering the patient. Despite the benign clinical picture in most cases, some reports have documented that infiltrates of SHML involving vital organ may cause death [2].

The diagnosis is often not considered in the first instance due to its rarity and histological similarity to other diseases. The disease process can either occur as purely extranodal or as a part of a generalized nodal disease. However, the histopathology for both nodal and extranodal disease is the same [2]. Approximately 43% of patients have at least one site of extranodal disease [2], [3].The extranodal form can affect a variety of regions in the head and neck, representing 22% of cases of extranodal Rosai–Dorfman disease [2]. There are very few numbers of reports of the disease affecting nasal cavity and paranasal sinuses [4], [5], [6], [7] or eye [8], [9].

We present what, to the best of our knowledge, is the first reported case of Rosai–Dorfman disease involving nodal, nasal cavity, paranasal sinuses, nasopharynx and orbit with facial asymmetry.

Section snippets

Case report

A 12-year-old boy with facial asymmetry was presented to the general surgery department with bilateral painless cervical and inguinal lymphadenopathy, nasal obstruction and proptosis for 10 months (Fig. 1). A full and complete physical examination showed no other abnormalities. Blood investigations showed an elevation of the erythrocyte sedimentation rate (30 mm/first hour) and elevated serum immunoglobulin (lgG = 30 and IgA = 2.3 g/l,). The complete blood pictures, renal function tests and Mantoux

Pathological findings

The infiltrate was composed of a large amount of mature plasma cells, histiocytes and small lymphocytes. Eosinophils and polymorphonuclear cells were absent. The histiocytes had abundant pale or eosinophilic cytoplasm with irregular indistinct cell borders and vesicular nuclei (Fig. 3). Multilobulated cells showed multiple nuclei, with atypical hyperchromatic ones which were occasionally seen. Mitotic figures were absent despite extensive search. Many of the histiocytes showed

Discussion

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) is a rare cause of lymph node enlargement in children. Most patients present in the first decade of life, with 80% of cases occurring in patients younger than 20 years. There is a slight male predominance. The disease occurs equally in blacks and whites and less commonly in Asians [2].

The cause of SHML is unknown, although an infectious agent, such as Epstein–Barr virus or herpesvirus, may be important in the pathogenesis

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