Nicol C, Quereux G, Renaut J-J, Renac F, Dreno B
Clinique dermatologique, CHU de Nantes, place Alexis-Ricordeau, Nantes cedex 01, France.
Ann Dermatol Venereol. 2011 May;138(5):405-8. doi: 10.1016/j.annder.2011.01.030. Epub 2011 Feb 26.
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.
多中心组织细胞增多症（MH）是一种病因不明的罕见组织细胞增多症形式，25%的病例与恶性肿瘤相关。在此，我们报告一例女性患者，通过MH我们得以诊断出胃癌。
一名74岁女性因手部丘疹和结节伴指间关节破坏性多关节痛及乏力前来就诊。对一个结节进行活检显示，浅表真皮有大量含嗜酸性细胞质的多核巨细胞浸润；免疫组化分析显示CD68染色阳性，确诊为MH。进一步的恶性肿瘤筛查使我们诊断出胃癌。皮肤病变在胃切除术后几周内消失，在随后的7年中未出现MH或恶性肿瘤复发。
报道的MH病例少于200例。这种非朗格汉斯细胞增生性组织细胞增多症是一种病因不明的罕见系统性疾病，临床特征为丘疹和结节伴破坏性多关节痛，组织学特征为皮肤、黏膜和滑膜的组织细胞性多核巨细胞的真皮增生。发现MH后应常规进行肿瘤筛查，因为25%的病例存在关联。然而，这两种疾病一般不会平行进展。在我们的病例中，术后几周内病变迅速消失表明MH是一种真正的副肿瘤综合征。
