Ferreli C, Atzori L, Zucca M, Pistis P, Aste N
Dermatology Department, Cagliari University via Ospedale 54-09124, Cagliari, Italy.
J Eur Acad Dermatol Venereol. 2004 Sep;18(5):599-602. doi: 10.1111/j.1468-3083.2004.00987.x.
Cutaneous leishmaniasis is an endemic protozoan infection in Sardinia, one of the major islands of the Mediterranean Basin. The main causative strain in this country is Leishmania infantum, which rarely involves mucocutaneous areas, but has the potential to cause visceral leishmaniasis. An atypical leishmaniasis involving the inferior lip of a 57-year-old female with Down's syndrome was observed at the Dermatology Department of Cagliari (italy). The diagnosis was mainly based upon histopathological examination, revealing intra- and extra-cellular leishmania amastigotes. The leishmania infantum zymodeme MON-111 was identified by isoenzymatic characterization. Laboratory investigations revealed a normal complete blood count and biochemistry profile, except for an inverted CD4/CD8 ratio. Treatment with meglumine antimoniate 60 mg/kg/day (Glucantime) intramuscularly for 15 days, followed by intralesional administration 1 ml weekly for 4 weeks led to complete recovery. No relapses were observed at 6-month follow-up. The unusual localization is likely to be a reflection of the uncommon site of inoculation of the protozoa, transmitted by bites from flying vectors. Nevertheless, the presence of Down's syndrome in our patient may have contributed to the atypical presentation by traumatic exacerbation of the lesion, due to repeated auto-induced microtraumas of the inferior lip accompanied by subclinical immunodeficiency. In fact, the specific immune response to Leishmania infection depends on a host-cell-mediated immune response, reported as defective in Down's syndrome patients. Differential diagnosis and early detection of the infection are necessary in order to start effective treatment and prevent more serious complications.
皮肤利什曼病是地中海盆地主要岛屿之一撒丁岛的一种地方性原生动物感染。该国主要致病菌株为婴儿利什曼原虫，很少累及黏膜皮肤区域，但有导致内脏利什曼病的可能。在意大利卡利亚里皮肤科观察到一例涉及一名57岁唐氏综合征女性下唇的非典型利什曼病。诊断主要基于组织病理学检查，发现细胞内和细胞外利什曼无鞭毛体。通过同工酶鉴定确定为婴儿利什曼原虫酶谱型MON-111。实验室检查显示全血细胞计数和生化指标正常，除了CD4/CD8比值倒置。用葡甲胺锑酸盐60mg/kg/天（葡醛锑钠）肌肉注射15天，随后每周皮损内注射1ml共4周，患者完全康复。6个月随访未观察到复发。这种不寻常的定位可能反映了由飞行媒介叮咬传播的原生动物接种部位不常见。然而，我们患者患有唐氏综合征可能因下唇反复自我诱发微创伤伴亚临床免疫缺陷导致病变创伤性加重，从而促成了这种非典型表现。事实上，对利什曼原虫感染的特异性免疫反应取决于宿主细胞介导的免疫反应，据报道唐氏综合征患者的这种免疫反应存在缺陷。为了开始有效治疗并预防更严重的并发症，进行鉴别诊断和早期感染检测是必要的。
