Picco P, Gattorno M, Vignola S, Barabino A, Marazzi M G, Bondi E, Pistoia V, Buoncompagni A
2nd Division of Pediatrics, G Gaslini Scientific Institute, Genoa, Italy.
Scand J Rheumatol. 1999;28(1):27-32.
We report a series of 22 children with idiopathic, drug unrelated erythema nodosum (EN) admitted to our Department. In 5 of them an history of streptococcal pharyngitis was referred; the remaining patients came to us with a diagnosis of "EN of unknown origin". Acute phase reactants, immunoglobulins, stool alpha1 antitrypsin, ANA, anti dsDNA antibodies and ANCA assay, chest roentgenogram, tuberculin test, and ophthalmologic assessment were performed in all patients. Etiologic diagnosis was made in 16 patients: Streptococcal pharyngitis (5 cases), chronic inflammatory bowel disease, IBD (3 cases), Behçet syndrome (2 cases), Yersinia enteritis (2 cases), infectious mononucleosis, atypical mycobacterial infection, immunodeficiency related infection, and SLE-like syndrome due to C4 deficiency (1 case each). We found oral/scrotal aphthae in 3 cases, gastrointestinal symptoms in 5 cases, arthritis in 3 cases. Acute phase reactants were positive in 16 patients without correlation to the underlying disease. Conversely, the increased alpha1 antitrypsin stool excretion and IgA serum concentration seemed to represent helpful indicators of IBD and Behçet syndrome, respectively. Proinflammatory cytokine pattern showed increased IL6 serum concentrations both in infectious and in non infectious disease-related EN, whereas a minor involvement of TNF was found in these patients.
我们报告了收治于我科的一系列22例特发性、与药物无关的结节性红斑（EN）患儿。其中5例有链球菌性咽炎病史；其余患者前来就诊时诊断为“不明原因的EN”。对所有患者均进行了急性期反应物、免疫球蛋白、粪便α1抗胰蛋白酶、抗核抗体（ANA）、抗双链DNA抗体和抗中性粒细胞胞浆抗体（ANCA）检测、胸部X线检查、结核菌素试验及眼科评估。16例患者明确了病因诊断：链球菌性咽炎（5例）、慢性炎症性肠病（IBD，3例）、白塞病（2例）、耶尔森菌肠炎（2例）、传染性单核细胞增多症、非典型分枝杆菌感染、免疫缺陷相关感染以及C4缺乏所致的系统性红斑狼疮样综合征（各1例）。我们发现3例有口腔/阴囊口疮，5例有胃肠道症状，3例有关节炎。16例患者急性期反应物呈阳性，与基础疾病无关。相反，粪便α1抗胰蛋白酶排泄增加和血清IgA浓度升高似乎分别是IBD和白塞病的有用指标。促炎细胞因子模式显示，在感染性和非感染性疾病相关的EN中，血清IL6浓度均升高，而这些患者中TNF的参与程度较小。
