Histiocytose multicentrique paranéoplasique

Abstract

Multicentric histiocytosis (MH) is a rare form of histiocytosis of unknown aetiology and is associated with a malignant neoplasm in 25% of cases. Herein, we report the case of a female patient in whom HM enabled us to diagnose gastric carcinoma.A 74-year-old woman consulted for papules and nodules on the hands associated with destructive polyarthralgia of the interphalangeal joints and asthenia. Biopsy of a nodule revealed infiltration of the superficial dermis by numerous multinucleated giant cells containing eosinophilic cytoplasm; immunohistochemical analysis showed positive staining for CD68, confirming the diagnosis of MH. Additional screening tests for malignancy enabled us to diagnose gastric carcinoma. The cutaneous lesions disappeared several weeks after gastrectomy and no recurrence of HM or malignancy was seen in the ensuing 7 years.Fewer than 200 cases of HM have been reported. This non-Langerhans proliferative histiocytosis is a rare systemic disorder of unknown aetiology characterized clinically by papules and nodules associated with destructive polyarthralgia, and histologically by dermal proliferation of histiocytic multinucleated giant cells of skin, mucous membrane and synovial membrane. Routine screening for neoplasia should be performed following the discovery of HM as an association is seen in 25% of cases. However, the two diseases do not generally progress in parallel. In our case, the rapid disappearance of lesions within a few weeks of surgery suggests that HM was a true paraneoplastic syndrome.