ABSTRACT
Madelung's disease, or benign symmetric lipomatosis, is an uncommon disorder that is characterized by massive symmetrical deposits of adipose tissue in the upper trunk, neck and head, and is usually associated with alcohol abuse; as such, patients usually complain of cosmetic issues. Historically, Madelung's disease is usually encountered in men between 30 and 60 years of age, and is more prevalent in the Mediterranean population. In this case study, we describe a rare case of Madelung's disease, in an Asian patient who presented with symmetrically located bilateral masses in the anterior neck, which grew progressively larger over a period of seven years.
Subject(s)
Humans , Male , Adipose Tissue , Alcoholism , Asian People , Head , Lipomatosis , Lipomatosis, Multiple Symmetrical , NeckABSTRACT
Relata-se o caso de um paciente portador da doença de Madelung, definida clinicamente pela presença de múltiplos acúmulos de tecido adiposo não encapsulados, usualmente envolvendo a região cervical e superior do tórax, de distribuição simétrica. O exame de imagem eleito para confirmação do diagnóstico foi tomografia computadorizada, por ser menos dispendiosa e mais acessível, comparando-se à ressonância magnética.
The authors report the case of a patient with Madelung's disease, which is clinically described as the presence of multiple and symmetric non-encapsulated masses of fatty tissue, usually involving the neck and the upper region of the trunk. Computed tomography was selected as the imaging method for diagnosis confirmation, considering its lower cost and higher availability as compared with magnetic resonance imaging.
Subject(s)
Humans , Male , Adult , Head and Neck Neoplasms , Lipomatosis, Multiple Symmetrical/diagnosis , Lipomatosis, Multiple Symmetrical/etiology , Head and Neck Neoplasms/complications , Adipose Tissue/pathology , Thorax/pathology , Head and Neck Neoplasms/surgery , Head and Neck Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
Benign symmetric lipomatosis (BSL) is characterized by the presentation of numerous symmetric non-tender fatty tumors in the neck, suboccipital region, proximal extremities and upper parts of the trunk, typically noted as a “pseudoathletic” appearance. The etiology of BSL remains unknown but mitochondrial mutations have been found in some patients. The mitochondrial cytopathy was suggested to be studied in all BSL patients especially those with neuropathy. We have presented a case of BSL with reverse fat distribution, which we named the “Pop-eye” appearance in which the fatty tumor was distributed at both the distal part of the upper extremities and the proximal part of the lower extremities. A study of the mitochondrial tRNA lysine gene did not reveal mutation at position 8344.
ABSTRACT
Benign symmetric lipomatosis is a very rare disease that's characterized by a symmetric accumulation of excessive amounts of adipocytes. The lesions are located mainly on the neck, trunk and proximal extremities. Although its etiology is unknown, it has been described to be associated with a heavy alcohol intake. A 51-year-old woman presented with a two year history of ill-defined soft masses on both mandibular areas. We made the diagnosis of benign symmetric lipomatosis according to the histopathologic examination. As a difference from other reports of benign symmetric lipomatosis, our patient was non-drinker for her past history. We present here a case of benign symmetric lipomatosis at an unusual predilection site and this developed in a non-drinker.
Subject(s)
Female , Humans , Middle Aged , Adipocytes , Extremities , Lipomatosis , Neck , Rare DiseasesABSTRACT
Benign symmetric lipomatosis is a rare disease of undetermined cause characterized by massive symmetric depositions of adipose tissue, predominantly on the neck, shoulder, back, and upper extremities. A 60-year-old man with chronic alcoholism was referred to our clinic for symmetric, skin-colored, non-tender masses on the posterior neck, both clavicular areas, abdomen, and back for about 10 years. He had been managed for tingling sensation on both hands in the neurologic department. Nerve conduction velocity studies were consistent with sensorimotor polyneuropathy and histopathologic findings revealed that the reticular dermis was replaced by normal uncapsulated mature fat cells. We report a rare case of benign symmetric lipomatosis associated with polyneuropathy.
Subject(s)
Humans , Middle Aged , Abdomen , Adipocytes , Adipose Tissue , Alcoholism , Dermis , Hand , Lipomatosis , Neck , Neural Conduction , Polyneuropathies , Rare Diseases , Sensation , Shoulder , Upper ExtremityABSTRACT
Benign symmetric lipomatosis is characterized by diffuse symmetric deposits of nonencapsulated fat, generally affecting the cervical and upper dorsal regions. There is a history of alcohol abuse in nearly all the cases. It is rarely reported in non-drinkers. Herein we report a 63-year-old man presenting with one year's history of multiple ill-defined symmetric soft mass on posterior neck, upper trunk and upper extremities without the history of alcohol abuse. We ad-ministered corticosteroid orally due to osteoarthritis of the knee for a long time.
Subject(s)
Humans , Middle Aged , Alcoholism , Knee , Lipomatosis , Neck , Osteoarthritis , Upper ExtremityABSTRACT
Benign symmetric lipomatosis is characterised by diffuse symmetric deposits of adipose tissue, predominantly on the neck, shoulder, back and upper extremities. We report a case of benign symmetric lipomatosis in a 61-year-old-man, which was first noted one year previously. It began growing rapidly about 3 months ago and was associated with alcoholic liver disease. Histopathological findings showed that the reticular dermis had been replaced by normal uncapsulated mature fat cells that were slightly increased in number in the fibrous connective tissue. He was treated with theophylline 300mg/day for two months.
Subject(s)
Adipocytes , Adipose Tissue , Connective Tissue , Dermis , Lipomatosis , Liver Diseases, Alcoholic , Neck , Shoulder , Theophylline , Upper ExtremityABSTRACT
Benign symmetric lipomat,osis is a rare disease of undetermined cause characterized by symmetric deposits of adipose tissue o:n the neck, upper trunk and abdomen. We report three cases of kienign symmetric lipomatosis, one of which is associated with alcoholic liver disease and the patient complained of a diffieulties in neck motion, All of them are about sixty year old men with history of frequent alcohol consumption. In histopathologic findings, the reticular dermis was completcly replaced by mature fat cells and there was a slight increase in vascular and fibrous elements.
Subject(s)
Humans , Male , Abdomen , Adipocytes , Adipose Tissue , Alcohol Drinking , Dermis , Lipomatosis , Liver Diseases, Alcoholic , Neck , Rare DiseasesABSTRACT
Benign symmetric lipomatosis is characterized by massive symmetric fat deposition, predominantly in the neck and shoulder girdle ares. We present a case of benign symmetric lipomatosis with alcoholic liver cirrhosis in a 54-year-old man. About two years ago, solitary skin-colored, non-tender swelling on the right upper arm was developed. The lesion had been aggravsted abruptly and rapidly in size and distribution since about one month ago. Histopathologic findings showed completely replaced reticular dermis and invaded subcutaneous tissue by mature fat cells.