Terheyden Patrick, Becker Jürgen C, Lurz Christa, Kahaly George J, Bröcker Eva B
Department of Dermatology, University of Würzburg, Würzburg, Germany.
J Dtsch Dermatol Ges. 2003 Jan;1(1):30-5. doi: 10.1046/j.1610-0387.2003.t01-1-02502.x.
Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. Patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis.
Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG lambda paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p.o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter.
Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61+, 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months.
Hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.
硬化性黏液水肿是一种病因不明的罕见疾病，其特征为黏蛋白沉积和皮肤硬化性硬结，与副蛋白血症相关。患者因活动受限和容貌毁损而逐渐丧失功能。硬化性黏液水肿的治疗是一项治疗挑战。抗疟药羟氯喹对网状红斑性黏液病有快速且可靠的疗效。
连续4例硬化性黏液水肿患者（2例女性，2例男性；中位年龄：50岁）接受了羟氯喹治疗，其中3例患者存在IgG λ副蛋白。治疗起始剂量为口服600 mg，持续10天，随后为400 mg，至少持续4周，之后为200 mg。
1例患者皮肤表现完全缓解，3例患者部分缓解，缓解持续时间分别为61个月以上、5个月和25个月。值得注意的是，3例患者在治疗的第一周感觉活动能力增强，皮肤硬度降低，这反映在皮肤厚度迅速减小。1例患者吞咽困难得到缓解，食管清除率恢复正常即为证据。副蛋白血症完全未受影响。副作用包括治疗19个月后出现1例视网膜电图异常，治疗3个月后出现1例白细胞减少。
羟氯喹是治疗硬化性黏液水肿的一种有效疗法，可快速且持久地缓解症状。
