Christy W C, Buckingham R B, Barnes E L, Prince R K
J Rheumatol. 1983 Aug;10(4):595-601.
Two patients with scleredema of Buschke are presented. The second patient developed scleredema after a febrile drug reaction. Biopsies of both involved and uninvolved skin were obtained for histologic examination and cell culture studies. Immunofluorescent studies of the biopsy specimens revealed staining with IgG, IgM and C3 at the dermal-epidermal junction in the involved skin of the first patient. This has not been previously reported. Cell culture studies revealed that fibroblasts from the involved skin produced more glycosaminoglycans (GAG) than uninvolved skin and most of the GAG produced was hyaluronic acid. Possible pathogenic mechanisms for this unusual condition are discussed.
本文报告了两例Buschke硬化性黏液水肿患者。第二例患者在药物热反应后出现硬化性黏液水肿。对受累皮肤和未受累皮肤均进行活检，以进行组织学检查和细胞培养研究。活检标本的免疫荧光研究显示，首例患者受累皮肤的真皮表皮交界处有IgG、IgM和C3染色。此前尚未有过此类报道。细胞培养研究表明，受累皮肤的成纤维细胞比未受累皮肤产生更多的糖胺聚糖（GAG），且产生的GAG大部分为透明质酸。文中讨论了这种罕见病症可能的致病机制。
