Varga J, Griffin R, Newman J H, Jimenez S A
Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA 19107.
J Rheumatol. 1991 Feb;18(2):259-63.
Induration of the skin develops in a majority of patients with the eosinophilia-myalgia syndrome associated with L-tryptophan, and bears striking clinical and histopathological resemblance to eosinophilic fasciitis (EF). These similarities have led to the suggestion that eosinophilia-myalgia syndrome and EF are the same disease. To study the relationship of eosinophilia-myalgia syndrome and EF, we ascertained the prevalence of L-tryptophan use in a cohort of patients with EF, and compared their clinical and laboratory findings to those of patients with eosinophilia-myalgia syndrome associated cutaneous involvement. None of 11 patients who were diagnosed as having EF between 1970 and 1989 used L-tryptophan containing preparations prior to the onset of their illness. Marked clinical and laboratory test differences were observed between patients with EF and eosinophilia-myalgia syndrome. Patients with eosinophilia-myalgia syndrome had a more acute onset, more severe symptoms, higher frequency of rash and of pulmonary, cardiac, gastrointestinal, neurologic, myopathic and thyroid involvement compared to patients with EF. Corticosteroid therapy resulted in improvement of cutaneous involvement in 88% of patients with EF but it was only partially successful in patients with eosinophilia-myalgia syndrome. Hospitalization and fatalities occurred only among patients with eosinophilia-myalgia syndrome. These observations demonstrate that eosinophilia-myalgia syndrome is a more severe disease with multisystemic involvement that can be clinically distinguished from EF. In contrast to eosinophilia-myalgia syndrome, EF is not associated with L-tryptophan ingestion.
大多数与L - 色氨酸相关的嗜酸性粒细胞增多性肌痛综合征患者会出现皮肤硬结,其在临床和组织病理学上与嗜酸性筋膜炎(EF)极为相似。这些相似之处使得有人认为嗜酸性粒细胞增多性肌痛综合征和EF是同一种疾病。为了研究嗜酸性粒细胞增多性肌痛综合征与EF的关系,我们确定了一组EF患者中L - 色氨酸的使用情况,并将他们的临床和实验室检查结果与伴有皮肤受累的嗜酸性粒细胞增多性肌痛综合征患者进行比较。1970年至1989年间被诊断为EF的11名患者在发病前均未使用过含L - 色氨酸的制剂。EF患者与嗜酸性粒细胞增多性肌痛综合征患者在临床和实验室检查方面存在明显差异。与EF患者相比,嗜酸性粒细胞增多性肌痛综合征患者起病更急,症状更严重,皮疹以及肺部、心脏、胃肠道、神经、肌肉和甲状腺受累的频率更高。皮质类固醇治疗使88%的EF患者的皮肤受累情况得到改善,但对嗜酸性粒细胞增多性肌痛综合征患者仅部分有效。住院和死亡仅发生在嗜酸性粒细胞增多性肌痛综合征患者中。这些观察结果表明,嗜酸性粒细胞增多性肌痛综合征是一种更严重的多系统受累疾病,在临床上可与EF区分开来。与嗜酸性粒细胞增多性肌痛综合征不同,EF与摄入L - 色氨酸无关。