Seskute G, Dapkute A, Kausaite D, Strainiene S, Talijunas A, Butrimiene I. Multidisciplinary diagnostic dilemma in differentiating Madelung’s disease — the value of superb microvascular imaging technique: A case report. World J Clin Cases 2021; 9(21): 6145-6154 [PMID: 34368337 DOI: 10.12998/wjcc.v9.i21.6145]
Corresponding Author of This Article
Goda Seskute, MD, Doctor, Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania. goda.seskute@santa.lt
Research Domain of This Article
Rheumatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Jul 26, 2021; 9(21): 6145-6154 Published online Jul 26, 2021. doi: 10.12998/wjcc.v9.i21.6145
Multidisciplinary diagnostic dilemma in differentiating Madelung’s disease — the value of superb microvascular imaging technique: A case report
Goda Seskute, Austeja Dapkute, Dominyka Kausaite, Sandra Strainiene, Andrius Talijunas, Irena Butrimiene
Goda Seskute, Irena Butrimiene, Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania
Austeja Dapkute, Clinic of Neurology and Neurosurgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania
Dominyka Kausaite, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania
Sandra Strainiene, Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius 01513, Lithuania
Andrius Talijunas, Institute of Dentistry, Faculty of Medicine, Vilnius University, Vilnius 01512, Lithuania
Author contributions: Seskute G was the rheumatologist who followed up with the patient; Seskute G, Dapkute A, and Kausaite D performed the patient’s data extraction, wrote the original manuscript, and reviewed the literature; Strainiene S, Talijunas A, and Butrimiene I reviewed and edited the manuscript; Butrimiene I was responsible for the revision of the manuscript for important intellectual content; All authors issued final approval for the version to be submitted and agreed to be accountable for all aspects of the work.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Goda Seskute, MD, Doctor, Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania. goda.seskute@santa.lt
Received: March 28, 2021 Peer-review started: March 28, 2021 First decision: May 7, 2021 Revised: May 13, 2021 Accepted: May 26, 2021 Article in press: May 26, 2021 Published online: July 26, 2021 Processing time: 114 Days and 13.1 Hours
Abstract
BACKGROUND
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.
CASE SUMMARY
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.
CONCLUSION
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.
Core Tip: Madelung’s disease, also known as benign symmetrical lipomatosis, is a rare disorder of fat metabolism resulting in unusual accumulation of subcutaneous fat deposits in different areas of the body and is mostly predisposed by alcohol abuse. The disease mimics and overlaps with other pathologies such as obesity, and oncological and connective tissue diseases and is associated with cirrhosis. High-end ultrasonography techniques have an important role in evaluating fatty tumors, and its importance for confirming diagnosis and follow-up for malignancy is unquestionable. A comprehensive approach to the patient and innovative ultrasound techniques are the key components for making an accurate diagnosis.
