Valicenti J M, Fleming M G, Pearson R W, Budz J P, Gendleman M D
Department of Dermatology, Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL.
J Am Acad Dermatol. 1991 Jul;25(1 Pt 1):54-8. doi: 10.1016/0190-9622(91)70174-z.
Five patients with the L-tryptophan-related eosinophilia-myalgia syndrome had a generalized eruption of flesh-colored papules. In all patients, histologic examination revealed a focal accumulation of mucin in the upper mid dermis, associated with increased dermal cellularity. The mucin was composed predominantly of hyaluronic acid, with small amounts of sulfated acid mucopolysaccharides. The cells within the lesion were fibroblasts. The lesions slowly regressed after L-tryptophan was discontinued. Proposed explanations for the L-tryptophan-related eosinophilia-myalgia syndrome have centered on contaminants, chemically related to L-tryptophan, introduced in the manufacturing process. Tryptophan metabolites have been linked with sclerotic cutaneous diseases but have not been previously implicated in cutaneous mucinoses.
5例与L-色氨酸相关的嗜酸性粒细胞增多性肌痛综合征患者出现了全身性肤色丘疹疹。所有患者的组织学检查均显示,真皮中上部有局灶性粘蛋白积聚,并伴有真皮细胞增多。粘蛋白主要由透明质酸组成,含有少量硫酸化酸性粘多糖。病变内的细胞为成纤维细胞。停用L-色氨酸后,病变缓慢消退。对于与L-色氨酸相关的嗜酸性粒细胞增多性肌痛综合征,提出的解释集中在制造过程中引入的与L-色氨酸化学相关的污染物。色氨酸代谢产物与硬化性皮肤病有关,但此前未被认为与皮肤粘蛋白病有关。