Shazzad M N, Azad A K, Abdal S J, Afrose R, Rahman M M, Haq S A
Dr Md Nahiduzzamane Shazzad, Medical Officer, Department of Rheumatology, Bangabandhu Sheikh Mujib MedicalUniversity (BSMMU), Dhaka, Bangladesh.
Mymensingh Med J. 2015 Jul;24(3):606-9.
Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histological peculiarity. No therapy is effective. In this communication we have presented a 54 year old man with scleredema successfully treated by PUVA and methotrexate. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment.
布施克硬肿病是一种病因不明的罕见结缔组织疾病，其特征为真皮增厚。它类似于系统性硬化症、嗜酸性筋膜炎和皮肤淀粉样变。硬肿病可能与既往发热性疾病、糖尿病或血液系统疾病史有关。它可分为三个临床组，每组有不同的病史、病程和预后。皮肤组织学特征为真皮增厚，由于真皮中粘多糖沉积增加，大胶原束之间的间隙增大。根据典型的临床特征和组织学特点可进行鉴别诊断。尚无有效的治疗方法。在本报告中，我们介绍了一名54岁患有硬肿病的男性患者，经补骨脂素加长波紫外线照射（PUVA）和甲氨蝶呤成功治疗。我们回顾了相关疾病、临床和组织病理学特征、病情发展及治疗反应。
