Capestrano Warrior in Portugal Madelung’s disease: cases report and review of the literature
Keywords:
Madelung’s disease, benign symmetric adipose massesl, Lanois-Bensaude syndrome, chronic alcoholism, surgical removalAbstract
Madelung’s disease is a rare disorder of unknown aetiology,
characterized by a progressive development of non capsulated
subcutaneous adipose tissue on the neck, shoulders, arms and
the upper parts of the trunk. Although of a benign nature, neck
or mediastinum involvement can cause compression syndromes of the trachea and superior vena cava causing dyspnea,
dysphagia and dysphonia. It predominantly affects men in the
30 to 60 years-old range, of Mediterranean origin and with a
history of alcohol abuse. The disorder can also be associated
with hyperlipoproteinemia, hyperuricemia, diabetes mellitus and
hypothyroidism. The only effective treatment is surgical removal or liposuction, but recurrences are common.
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References
Feliciani C, Amerio P: Madelung’s disease: inherited from a Mediterranean population? N Engl J Med 1999; 340: 1481.
Kratz C, Lenard HG, Ruzicka T Gartner J: Multiple symmetrical lipomatosis: an unusual cause of childhood obesity and mental retardation. Eur J Paediatr Neurol 2000; 4: 63-67.
Bulum T, Duvnjak L, Car N, Metelko Z Madelung’s disease: Case report and review of the literature. Diabetologia Croatica 2007; 36-2: 25- 30.
Josephson GD, Selafani AP, Stern J. Benign symmetric lipomatosis (Madelung’s disease). Otolaryngol Head Neck Surg 1996; 115: 170.
Enzi G. Multiple symmetrical lipomatosis. An updated clinical report. Medicine (Baltimore) 1984; 63: 56.
Brodie BC: Lectures illustrative of various subjects in pathology and surgery. London: Longman, Brown, Green, and Longman, 1846: 275-282.
Otto Wilhelm Madelung: Veber den Fetthals; Arch. Klin. Chir. 1888; 37: 106-130.
Borges A, Torrinha F, Lufkin RB, Abemayor E: Laryngeal involvement in multiple symmetric lipomatosis. The role of computed tomography in diagnosis. Am J Otolarylgol 1997; 18: 127.
Vargas-Díez E, Daudén E, Junes-Callero M, García-Díez A: Madelung’s disease involving the tongue. J Am Acad Dermatol 2000; 42: 511-513.
Crepaldi G: Metabolic abnormalities in multiple symmetric lipomatosis: elevated lipoprotein lipase activity in adipose tissue with hyperalphalipoproteinaemia. J Lipid Res 1983; 36: 187.
Nielsen S, Levine J, Clay R, Jensen MD: Adipose tissue metabolism in benign symmetric lipomatosis. J Clin Endocrinol Metab 2001; 86: 2717-2720.
Tizian C, Berger A, Vykouph K: Malignant degeneration in Madelung’s disease. Br J Plast Surg 1983; 36: 187.
Guastella C, Borsi C, Gibelli S, Della Berta LG: Madelung’s lipomatosis associated with head and neck malignant neoplasia: a study of 2 cases. Otolaryngol Head Neck Surg 2002; 126: 191-192.
Klopstock T, Naumann M, Schalke B et al: Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. Neurology 1994; 44: 862-866.
Berkovic SF, Andermann F, Shoubridge EA et al: Mitochondrial dysfunction in multiple symmetrical lipomatosis. Ann Neurol 1991; 29: 566-569.
Morinaka S, Sato T, Miyoshi H, Iwashita K. A case of multiple symmetrical lipomatosis (Madelung’s disease). Auris Nasus Larynx 1999; 26: 349-353.
Jonh DG, Fung HK, Van Hasselt CA, King WW: Multiple symmetrical lipomatosis in the neck. Eur Arch Otorhinolaryngol 1992; 249: 277-288.
Kohan D, Miller PJ, Rothdtein SG, Kaufman D: Madelung’s disease: case reports and literature review. Otolaryngol Head Neck Surg 1993; 108: 156-159.
Lieber CS: Ethanol metabolism. Cirrhosis and alcoholism. Clin Chim Acta1997; 257: 96-102.
Morelli F: Alcoholism as a trigger of multiple symmetric lipomatosis? Dermatol Venereol 2003; 17(3): 367-369.
Naumann M, Sckalke B, Klostock T el al. Neurological multisystem manifestation in multiple symmetric lipomatosis: a clinical and electrophysiological study. Muscle Nerve 1995; 18: 693-699.
Boozan JA, Maves MD, Schuller DE: Surgical management of massive benign symmetric lipomatosis. Laryngoscope 1992; 102: 94.
Saiz-Hervas E, Martin Llorens M, Lopez Alvarez J: Peripheral neuropathy as the first manifestation of Madelung’s disease. Br J Dermatol 2000; 143: 684.
Ruzicka T, Vieluf D, Landthaler M, Braun-Falco O: Benign symmetric lipmatosis Launois-Bensaude. Report of ten cases and review of the literature. J Am Acad Dermatol 1987; 17: 663-674.
Ujpal M, Nemeth S, Reichwien A et al. Long term results following surgical treatment of benign lipomatosis (BSL). Int J Oral Maxillofac Surg 2001; 30: 479.
Faga A, Valdatta LA, Thione A, Buoro M: Ultrasound-assisted liposuction for the palliative treatment of Madelung’s disease: a case report. Aesthetic Plast Surg 2001; 25: 181.
Leung NW, Gaer D, Beggs D, Kark A et al. Multiple symmetrical lipomatosis. Effect of oral salbutamol. Clin Endocrinol 1987; 27: 601
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