Seskute Goda, Dapkute Austeja, Kausaite Dominyka, Strainiene Sandra, Talijunas Andrius, Butrimiene Irena
Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania.
Clinic of Neurology and Neurosurgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania.
World J Clin Cases. 2021 Jul 26;9(21):6145-6154. doi: 10.12998/wjcc.v9.i21.6145.
Madelung's disease, also known as multiple symmetrical lipomatosis, is a rare, underrecognized disorder of fat metabolism that results in unusual accumulation of subcutaneous fat deposits around the neck, shoulders, upper arms, trunk, hips, and upper thighs. Our case demonstrates the importance of differential diagnosis and the value of a superb microvascular imaging technique for suspecting and confirming Madelung's disease. Timely diagnosis and alcohol abstinence could prevent the progression of growing fatty masses and prevent surgery.
A 62-year-old male was admitted to the Rheumatology center complaining of symmetric subcutaneous tumors in the area of the parotid and submandibular salivary glands, small soft masses in the occiput and upper third of the forearm, rashes on calves. A high titer of rheumatoid factor and low concentrations of serum complements were detected. The high-end ultrasound and magnetic resonance imaging examinations of all affected areas of the soft tissues showed predominantly adipose tissue (lipomas) without suspicion of liposarcoma. The biopsy from the small salivary gland revealed no pathology. After evaluating the patient's clinical presentation (symmetrical lipomatosis, cirrhosis, gynecomastia, anemia, hyperuricemia), Madelung's disease, type I, along with the psoriatic rash and psoriatic arthritis and secondary liver cirrhosis were established.
Madelung's disease consists of many co-occurring disorders imitating and overlapping with other conditions. Ultrasonography is the first choice for suspecting and confirming symmetrical lipomatosis.
马德隆氏病，又称多发性对称性脂肪瘤病，是一种罕见的、未被充分认识的脂肪代谢紊乱疾病，可导致颈部、肩部、上臂、躯干、臀部和大腿上部周围皮下脂肪异常堆积。我们的病例证明了鉴别诊断的重要性以及一种高超的微血管成像技术在怀疑和确诊马德隆氏病方面的价值。及时诊断和戒酒可防止脂肪块增大，并避免手术。
一名62岁男性因腮腺和颌下唾液腺区域对称皮下肿瘤、枕部和前臂上三分之一处小软块、小腿皮疹入住风湿病中心。检测到高滴度类风湿因子和低浓度血清补体。对所有受影响软组织区域进行的高端超声和磁共振成像检查显示主要为脂肪组织（脂肪瘤），未怀疑脂肪肉瘤。小唾液腺活检未发现病变。在评估患者的临床表现（对称性脂肪瘤病、肝硬化、男性乳房发育、贫血、高尿酸血症）后，确诊为I型马德隆氏病，同时伴有银屑病皮疹、银屑病关节炎和继发性肝硬化。
马德隆氏病由许多与其他病症相似和重叠的并发疾病组成。超声检查是怀疑和确诊对称性脂肪瘤病的首选方法。
