[Destombes Rosaï Dorfman syndrome: description of one case and diagnostic challenges in the tropical setting].
Destombes Rosaï Dorfman (DRD) syndrome is form of nonlangerhans cell sinus histiocytosis. The main symptoms are cervical adenopathy, fever and fluctuating hepatosplenomegaly. It can be confused with ganglionary tuberculosis especially in our region where tuberculosis is common. This report describes a case of Destombes Rosaï Dorfman syndrome in a 40-year-old woman from Senegal. The main presenting symptom was the presence of massive tumour-like lesions on the neck with altered general condition and fever. Based on these clinical findings, ganglionary tuberculosis was suspected and presumptive treatment was initiated in the local hospital. However further workup failed to confirm the diagnosis and the patient was transferred to the Internal Medicine Department. Clinical examination in our service revealed the presence of extensive adenopathy in the supraclavicular, axillary, and inguinal regions. Laboratory tests demonstrated a nonspecific inflammatory syndrome. Abdominal ultrasonography depicted extensive mesenteric and para-aortic adenopathy. Chest x-ray showed bilateral and asymmetric mediastinal adenopathy. Medullogram findings were normal. Histology confirmed DRD syndrome. DRD syndrome is rare disease of unknown aetiology. In tropical areas differential diagnosis with ganglionary tuberculosis, lymphoma, and reactive hemophagocytic syndrome can be challenging. Lymph node biopsy should be performed in all patients presenting fever and polyadenopathy.
Ndongo
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Clinique Médicale I, CHU Le Dantec. sndongo_medinterne@yahoo.fr
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Diouf
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D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005334:Fever / Q000209:etiology; D015618:Histiocytosis, Sinus / Q000175:diagnosis*; D006801:Humans; D008199:Lymphadenitis / Q000209:etiology; D012675:Senegal; D014329:Tropical Climate; D014388:Tuberculosis, Lymph Node / Q000175:diagnosis