Ozkaya-Bayazit E, Kavak A, Güngör H, Ozarmagan G
Department of Dermatology, Istanbul Medical Faculty, Istanbul University, Turkey.
Int J Dermatol. 1998 Dec;37(12):949-54. doi: 10.1046/j.1365-4362.1998.00600.x.
Severe and therapy-resistant pruritus is the most prominent feature of macular (MA) and lichen (LA) amyloidosis that leads to further amyloid deposition by recurrent frictional trauma to the epidermis. Of the various therapeutic modalities with variable success, the most encouraging and beneficial effect has been observed with topical dimethyl sulfoxide (DMSO) therapy. In a previous study, we achieved marked clinical improvement in nine of 10 patients in a daily treatment regimen over 6-20 weeks, but relapses occurred in the post-treatment follow-up period. The aims of this study are to investigate whether the patients would benefit from intermittent therapy and to determine the optimal application interval of DMSO to maintain the relief of symptoms.
Thirteen patients with histopathologically verified cutaneous amyloidosis (five MA, two LA and six biphasic) were enrolled in the study. They were treated once daily with a 50 or 100% DMSO solution until pruritus disappeared. Then, DMSO was applied at increasing intervals until the widest effective application interval for maintenance of relief was reached. Patients were regularly followed-up by a scoring system for pruritus, papules, and pigmentation, control biopsies, photographs, blood biochemistry, and side-effects.
The mean time required for the disappearance of pruritus was 4.1 weeks. Remarkable flattening of the papules was achieved after an average therapy period of 9 weeks. After a total therapy period of 6.5 months, a nearly 50% remission in pigmentation and >70% flattening of papules were achieved. The widest effective DMSO application interval was 8.6 days. The side-effects of therapy were contact urticaria, desquamation, burning sensation, and garlic-like breath odor, which were more prominent with the higher concentration of DMSO. In interval therapy, side-effects were tolerated more easily than in daily therapy. No reduction of amyloid deposits was revealed in control biopsies.
Locally applied DMSO can break the vicious "pruritus-amyloid deposition-pruritus" cycle in patients with MA and LA. In addition to its daily use, interval therapy seems to maintain this effect and enables patients to tolerate side-effects more easily.
严重且难治性瘙痒是黄斑型（MA）和苔藓型（LA）淀粉样变的最突出特征，反复摩擦表皮会导致进一步的淀粉样蛋白沉积。在各种疗效各异的治疗方式中，局部使用二甲基亚砜（DMSO）治疗取得了最令人鼓舞且有益的效果。在先前的一项研究中，我们对10例患者采用每日治疗方案，在6至20周内有9例患者取得了显著的临床改善，但在治疗后的随访期出现了复发。本研究的目的是调查患者是否能从间歇治疗中获益，并确定DMSO的最佳应用间隔以维持症状缓解。
13例经组织病理学证实的皮肤淀粉样变患者（5例MA、2例LA和6例双相型）纳入本研究。他们每天用50%或100%的DMSO溶液治疗一次，直至瘙痒消失。然后，逐渐延长DMSO的应用间隔，直至达到维持缓解的最宽有效应用间隔。通过瘙痒、丘疹和色素沉着评分系统、对照活检、照片、血液生化检查及观察副作用对患者进行定期随访。
瘙痒消失所需的平均时间为4.1周。平均治疗9周后丘疹明显变平。总治疗期6.5个月后，色素沉着减轻近50%，丘疹变平超过70%。DMSO的最宽有效应用间隔为8.6天。治疗的副作用有接触性荨麻疹、脱屑、烧灼感和蒜样口气，DMSO浓度越高副作用越明显。在间歇治疗中，副作用比每日治疗更容易耐受。对照活检未发现淀粉样蛋白沉积减少。
局部应用DMSO可打破MA和LA患者中“瘙痒-淀粉样蛋白沉积-瘙痒”的恶性循环。除每日使用外，间歇治疗似乎能维持这种效果，并使患者更容易耐受副作用。
