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Case Report

Multiple Symmetrical Lipomatosis with


Sabha Memon1,
Mukesh Sriwastava2, Involvement of Tongue
Rajeev Khanna3,
Mohd Ashraf Ganie 4 Abstract
1,2,3,4 Lipomatosis is a condition characterized by multiple noncapsulated areas of fat
Department of
Medicine Fortis Hospital
accumulation with histology consistent with adipose tissue. Involvement of tongue
Amritsar and Department of along with multiple symmetrical lipomatosis is extremely rare. Here we report a case
Endocrinology, Metabolism of multiple symmetrical lipomatosis involving tongue and presenting as macroglossia.
and Diabetes All India
Institute of Medical Keywords: Lipomatosis, Macroglossia, Madelung disease, Symmetrical lipomatosis.
Sciences, New Delhi, India.
Introduction
Correspondence to:
Dr. Mohd Ashraf Ganie, Lipomatosis is a condition characterized by multiple areas of fat accumulation, the
Department of areas being noncapsulated and histology consistent with adipose tissue.1 Multiple
Endocrinology and symmetrical lipomatosis (MSL) is characterized by formation of multiple
Metabolism, All India
Institute of Medical
noncapsulated lipomas in a symmetrical distribution with sparing of distal arms and
Sciences, New Delhi, India. legs.2 MSL with involvement of tongue is extremely rare. No case has been reported
in the literature till now. A case of MSL with macroglossia because of involvement of
E-mail Id: ashraf.endo@
gmail.com
tongue is presented.

Case Report
A 43-year-old lady was detected to have diabetes mellitus at the age of 30 years on
the basis of weight loss, osmotic symptoms and random venous plasma glucose of
280 mg/dL. She was put on medical nutrition therapy and oral anti-diabetic agents
for initial 5 years, and subsequently was put on two doses of premixed 30:70 insulin 5
units twice daily. Fasting plasma glucose would remain around 140–160 mg% and
two-hour post-prandial around 200 mg%. She had gradually noticed swellings around
neck and front of chest more on right side. She also had noticed difficulty in
breathing. She was nonalcoholic and had no apparent chronic complications of
diabetes mellitus. She had family history of diabetes mellitus in father and two
siblings. She was menstruating regularly. Examination revealed height 149 cm,
weight 47 kg with BMI of 21.17 kg/M2, blood pressure was 124/100 mmHg spine. She
had diffuse swelling around the front of neck giving the appearance of “double chin”.
In addition she also had diffuse swellings in supraclavicular, deltoid areas and front of
the chest (Fig. 1). She also had thickened lower lip and macroglossia with teeth marks
over it. Examination of nervous system revealed absent bilateral ankle and knee
jerks. Fundus examination revealed bilateral non-proliferative retinopathy. Rest of
the systemic examination was normal. Autonomic function revealed loss of
parasympathetic and normal sympathetic activity. Investigations revealed normal
complete blood count with ESR of 50 mm/hr, routine tests for liver and kidney
How to cite this article: functions were normal as was serum calcium and phosphorus. Serum lipids revealed
Memon S, Sriwastava M, total cholesterol of 199 mg/dL, low-density lipoprotein of 120 mg/dL, high density
Khanna R et al.Multiple lipoprotein of 47 mg/dL, triglycerides of 164 mg/dL and very-low-density lipoprotein
Symmetrical Lipomatosis
of 32 mg/dL. Serum uric acid was 2.4 mg/dL, Thyroid function and 8 AM cortisol was
with Involvement of Tongue.
J Adv Res Med 2016; 3(2&3): normal. Computed tomography revealed lipomatosis of neck, upper chest, without
8-10. any evidence of mediastinal extension. Biopsy was consistent with lipomatosis. A
diagnosis of multiple systemic lipomatosis (Madelung diseases) with macroglossia
ISSN: 2349-7181 was made and patient was put on insulin and oral anti-diabetic agents. Metabolic
control was achieved and patient was planned for excision of lump in front of neck
but did not follow.

© ADR Journals 2016. All Rights Reserved.


J. Adv. Res. Med. 2016; 3(2&3) Memon S et al.

Discussion Autonomic neuropathy is common in these patients


which contributes to high mortality in them.11 The
The first description of MSL dates back to the middle of disease being more prevalent in men and alcohol intake
19th century when Sir Benjamin Brodie described two has been reported in most of the patients.9 Local
patients in 1846.3 The disease was named after abnormalities in the form of upper airway compression
Madelung in 1888 after the author described three and metabolic abnormalities in the form of
more patients with sub mental fat deposition.4 The hyperlipidemia, hyperglycemia and hyperuricemia have
disease has mostly been reported from Mediterranean been reported in these patients.5 Our patient had
and European subjects. The reported cases of MSL with diabetes mellitus and hyperlipidemia without any
involvement of tongue are rare. Only three cases have evidence of hyperurecemia.
been described in the literature till now,5-8 and this is
the first case described from India. Depending on the The incidence of CAD is low in these patients because of
appearance of lipomatosis, two types have been increased high-density lipoprotein production and rapid
described, viz., type-1 if fatty masses maintained the clearance of triglycerides-rich lipoprotein lipase from
distinct, will circumscribed character protruding from circulation. An increased lipoprotein lipase activity in
the body and type-II if lipomatous tissue involved adipose tissue has been reported in these patients that
diffusely the subcutaneous fatty layer giving the may contribute to the protein lipoid profile in these
appearance of simple obesity.9 The disease is prevalent patients.12,13 High uric acid has mostly been reported in
in males and so are the reported cases of MSL with patients with high alcohol ingestion and our patient did
involvement of tongue.6-8 The distribution of lipomatous not have and was not alcoholic. Upper air way
tissue has mostly been reported in neck, dorsal, deltoid, obstruction contributes significantly to morbidity in
mammary regions, abdomen, and proximal segments of these patients as in our case and most of the patients
limbs. These situations are same as distribution of need surgery for relief. Involvement of tongue leads to
brown adipose tissue (BAT) in fetus and it is believed further disturbance in respiration as was the case in our
that growth of lipomatous cells stem from BAT patient.
precursor.10,11

Figure 1.Symmetrical Fat Deposit in Lower Lip, Submental Area, Neck, Supraclavicular,
Deltoid and Upper Chest (Arrows)

In summary, the present case description is that of a in women. To our knowledge, this is the first such case
young lady with multiple symmetrical lipomatosis with reported from India.
involvement of lower lip and tongue, who also had
diabetes mellitus and hyperlipidemia. The case scenario Conflict of Interest: None
is interesting in view of rarity of the disorder, especially

9 ISSN: 2349-7181
Memon S et al. J. Adv. Res. Med. 2016; 3(2&3)

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Date of Submission: 12th Sep. 2016
8. Vargas Diez E, Dauden E, Caballero MJ et al.
Date of Acceptance: 27th Sep. 2016

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