Yonago Acta medica 2005;48:101–106

Benign Symmetric Lipomatosis: A Patient Report
Yoshiyuki Tanji, Noriko Ikeda, Masami Shibata, Isamu Kodani, Kazuhito Tobashi,Kazunori Kidani, Fumihiro Otake, Kazuhiko Tanio and Kazuo Ryoke
Division of Oral and Maxillofacial Biopathological Surgery, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Faculty of Medicine, Yonago,
683-8504 Japan

Benign symmetric lipomatosis (BSL) is a disease characterized by symmetrical anddiffuse adipose tissue deposits on the neck, shoulder, chest, abdomen and groin. It is
comparatively common in the Mediterranean basin, but rare in Japan. We report on
a 53-year-old male patient with BSL along with a review of the literature. The lesions
were distributed bilaterally in the submandibular, neck and upper back region. Thispatient had consumed an excessive amount of alcohol with elevated liver enzymes including aspartate aminotransferase (133 IU/L), alanine aminotransferase (159 IU/L) andgamma-glutamyl transpeptidase (355 IU/L). The liver function was normalized after a
1-year restriction of alcohol consumption through life management. Tumors in the posterior and anterior cervical regions were resected. The patient is still in a good condition
at 3 years and 5 months after the operation.
Key words: alcoholic hepatopathy disease; benign symmetric lipomatosis

ings in the right submandibular and right lumbar
regions. Only tumors in the right lumbar region(2.5 cm and 1 cm in diameter, respectively) wereexcised, and diagnosed as lipoma histopathologically, while follow-up was performed on the submandibular swelling. He visited an internist in
early 2000 when he was 53 years old, because hestarted to notice a swelling in the right anterior region of the neck. He was referred to our hospital.
He began to drink at the age of 18. His drinking
increased gradually from the age of approximately 32, eventually drinking 2L of Japanese sake(ca. 15% alcohol) a day. Though the amount ofdrinking was gradually reduced thereafter, he still
drank about 1L of beer everyday. His sake index
was 183 [sake index: the number of units of dailysake consumption (1 unit = 27 g alcohol) multi-Benign symmetric lipomatosis (BSL) was firstreported in 1846 by Brodie et al. (1846) as a comparatively rare disease characterized by diffuse
symmetric deposits of fat. Etiology of BSL has
not been clear. The neck is affected most often,
but the shoulder, chest, abdomen and inguinal
regions can also be involved (Enzi, 1984). Although the BSL is comparatively common in the
Mediterranean basin, it is rare in Japan. Here, wereport a patient of BSL in the cervical region withalcoholic liver disease.

Patient Report
In 1993, a 46-year-old male visited a surgicalclinic at a general hospital, complaining of swell-Abbreviations: ALT, alanine aminotransferase; AST, aspartate aminotransferase; BSL, benign symmetric lipomatosis;
CT, computed tomography; γ-GTP, gamma-glutamyl transpeptidase; HE, hematoxylin and eosin; LDH, lactate dehydrogenase; TTT, thymol turbidity test; ZTT, zinc sulfate turbidity test101

Y. Tanji et al.

Fig. 1. Appearance of the patient at the1st visit.
A: The front side shows diffuse
masses in the cervical and submandibular regions.
B: The back side has masses in theneck and back regions, looking
like the buffalo hump.

AB

plied by number of years of drinking] (Miyaharaet al., 1981).
Physical examination revealed a numberof masses: one (60 × 100 mm) in the right submandibular region, one (45 × 45 mm) in the left
submandibular region, one (50 × 50 mm) in thesubmental region, one (30 × 45 mm) in the anterior neck, one (30 × 50 mm) in the left lateralneck, four (57 × 90 mm, 25 × 25 mm, 40 × 40mm and 50 × 60 mm) in the posterior neck andtwo (60 × 50 mm and 70 × 100 mm) in the upper back regions (Figs. 1A and B). These masses
were moderately hard but without tenderness,and their margins were indiscernible. Whenhe visited our hospital, his height was 161.5 cm
with a body weight of 64 kg. His cervical region
appeared like the buffalo hump. His plateletlevel was 75,000/μL. In liver function tests, thelevels were: aspartate aminotransferase (AST),
133 IU/L; alanine aminotransferase (ALT), 159
IU/L; gamma-glutamyl transpeptidase (γ-GTP),
355 IU/L; thymol turbidity test (TTT), 19.6 KU;
zinc sulfate turbidity test (ZTT), 11.2 KU; lactate
dehydrogenase (LDH), 236 IU/L and uric acid,8.4 mg/dL (Table 1). CT (computed tomography)
showed swelling in the bilateral cervical regions,the posterior region of the neck, and the supraclavicular region. His CT value for the swellings
was –100 Hounsfield units, the same as that for
fatty tissues. The inner tissues of the masses werecomparatively uniform, and no ﬁndings suggested

Table 1. Clinical laboratory ﬁndings
Examination
Blood countWhite blood cell
Red blood cell
Hb
Hematocrit
Mean corpuscular volume
Mean corpuscular Hb
Mean corpuscular Hbconcentration
PlateletLevel
(/μL)
(/μL)
(g/dL)
(%)
(fL)
(pg)

5.5 × 103
4.18 × 106
15.5
H
45.6
H
109.1
H
37.2
H(%)
(/μL)

34.1
75 × 103 L

Biochemical test
Na(mEq/L)
K(mEq/L)
Cl(mEq/L)
Blood urea nitrogen(mEq/L)
Cr(mg/dL)
Ca(mg/dL)
Aspartate aminotransferase (IU/L)
Alanine aminotransferase (IU/L)
Gamma-glutamyltranspeptidase(IU/L)
Lactate dehydrogenase(IU/L)Creatine kinase(IU/L)
Thymol turbidity test(KU)
Zinc sulphate turbidity test (KU)
Uric acid(mg/dL)
Urine test
Urinometry
Urine sugar
Urine protein1414.4
105
100.75
8.8
133
159
355
236
84
19.6
11.2
8.4

HHH
H
H
H

1.022
Negative
Negative

H, higher than normal levels; L, lower than normal levels

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A patient with lipomatosis

Fig. 2. Radiographic images of masses
A: A CT image visualizes hyperplasia of the fatty tissue in the posterior region of the neck (arrow).

A

B: A T2-emphasized magnetic resonance imaging shows high signals at the posterior subcutaneous
region of the neck and the upperback region (arrows).

Bmalignancy. The borders with normal tissue werenot clear (Fig. 2A). Ultrasonic inspection showed
thick connective tissues in the submandibularand neck regions. A low net-shaped signal area
was visible. T2-emphasized magnetic resonanceimaging presented high signals in the neck, submandibular regions and posterior regions of theneck, bilaterally. The boundary of lesions was notclear in each region (Fig. 2B). On July 19, 2000,
the right submandibular lesion was subjected tobiopsy. Histopathologically, it was diagnosed aslipomatosis due to hyperplasia of mature, nonatypical fat cells (Figs. 3A and B). A diagnosis of
BSL was determined from these results.

Moderate levels of hepatopathy were foundin the 1st routine blood test (Table 1), and the patient was referred to the Second Clinic of Internal
Medicine at our hospital for further evaluation.
Being diagnosed with alcoholic liver disease at
the clinic, alcohol restriction was begun throughlife habit guidance for improvement of hepaticfunction.
On July 19, 2001 when he laid down to rest,
he had shortness of breath and bilateral swelling in the upper arms. Vascular phlebography
revealed no arctation of the subclavicular vein oneither side, nor displacement toward the outsideof the brachiocephalic vein. After improvement

ABFig. 3. Histopathological ﬁgures obtained at biopsy. Hyperplasia of matured fat cells with no atypical formation isrecognized diffusely.
A: HE stain. Bar = 1 mm.
B: HE stain. Bar = 50 μm.

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Y. Tanji et al.

of hepatic function (AST: 79 IU/L, ALT: 80 IU/
L, γ-GTP: 161 IU/L, TTT: 4.1 KU and ZTT: 5.7
KU), breathlessness and swelling of the upperarms, tumors of the posterior and anterior regionsof the neck were surgically removed on August23, 2001. A mass in the right posterior neck was
not resected for the change of the position. Grossexamination of a fatty mass resected from the left
posterior neck region revealed diffuse inﬁltration
into the surrounding tissue with an unclear border (Fig. 4). The excised tumor was histopathologically diagnosed as lipomatosis on the basisof lobulated hyperplasia of mature adipocytes.
Since the operation, the patient has refrained fromdrinking. Up until 3 years and 5 months postoperation, no additional masses have appeared,
and no enlargement of the non-resected masses
have been detected.

Fig. 4. A tumor specimen excised from the left posterior region of the neck. Bar = 1 cm.

and its boundary with the surrounding tissue isvague; it also inﬁltrates the muscle and the retroperitoneal region, diffusely. This often leads torecurrence after excision. Type II is symmetrical,
diffuse lipomatosis appearing in adult life, primarily localized in the neck region but occasionally accompanied by symmetrical lipomatosis inother parts of the body. This condition is hereditary. Type III is multiple lipomatosis consistingof usually numerous, small, well-deﬁned, subcutaneous lipomas, mainly localized to the limbs,
especially the forearms, but occasionally also tothe trunk. The tumors which are surrounded byﬁbrous capsules, are easy to enucleate and neverrecur in the same localization although new elements will often appear elsewhere. This condition is also considered to be hereditary. According to Carlsen’s classification, our patient may
be Type II, the symmetrical, diffuse type due tosymmetrical bilateral lesions in the neck and upper back and presenting a buffalo hump, though
its hereditary character was not recognized.
There are some reports that these lesions havebeen seen in the chest, abdomen and other places.
A favorite site of lipomatosis Type II is in the cervix, also seen in the present patient, though theends of the limbs and the lower half of the bodyare not usually invaded. There are other reportsthat lipomatosis occurs in the tongue though oral
area lesions are rare (Vargas-Dies et al., 2000;

Discussion
Multiple lipomatosis was first reported by Brodie (1846). Madelung described 33 patients with
“Madelung’s disease” (1888). Since then, thisdisease had been called Madelung’s diffuse symmetric lipomatosis, but most recently, cases havebeen reported as BSL. Approximately 400 patients have been described in the literature so far(Harsch et al., 2004). BSL predominantly affects
white males (male:female ratio, 15:1). The fat deposits ﬁrst appear after the age of 20 and becomesmost common between the ages of 30 and 60(Ruzicka et al., 1987). The highest prevalence isin the Mediterranean basin (reported incidence inItaly, 1 in 25,000 males) (Parmar et al., 1996). In
Japan, after the ﬁrst report by Okuma (1941), BSL
appeared from time to time, showing a tendency
towards increase. Although no classiﬁcation forlipomatosis is established at present, Carlsen’sclassiﬁcation which clinically organizes systemic
benign lipomatosis into 3 types is generally accepted (Carlsen et al., 1978). Type I is diffuse
and congenital lipomatosis. It develops mainly inthe body trunk with no inheritable characteristics,
104

A patient with lipomatosis1991), dietetic treatment, (Ruzicka et al., 1987)
including forbidding alcoholic intake, as othermodalities of treatment. In this patient, refraining from alcohol has helped keep a healthyhepaticcondition. Also, during the 3 year and 5 monthfollow up, no additional masses were detected.
Thus, the etiology of BSL might be associatedwith alcoholic hepatopathy.

Sato et al., 2004). There may be various factorsthat cause lipomatosis, which have not yet beenclariﬁed. Alcoholic liver disease (Berkovic et al.,
1991; Nobukiyo et al., 1997), diabetes (Springer etal., 1972), hyperlipemia (Greene et al., 1970), gout(Taylor et al., 1961), hypothyroidism (Hugo et al.,1966) and hyperuricemia (Kawamoto et al., 1985)
are often accompanying complications. Etiologically, liver dysfunction can relate to a long historyof drinking and this, coupled with hyperuricemia,
could be related to our patient. Miyahara et al.
(1981) reported that a sake index of over 50 tends
to cause hepatopathy due to alcohol consumption.
Our patient’s sake index was 183, and his liver
seemed to be damaged.
One report explained that the fatty deposition of this disease is not systemic but localized,
and that a decrease of adipose tissue in the lesion
by means of lessening excessive weight is apparently low compared to normal regions (Greeneet al., 1970), so it is suggested that this disease
has no direct relation to obesity. There is anotherreport that these lesions may grow gradually forseveral years after onset and then stop at somepoint, becoming a fixed size in many patients.
No recurrence has been observed in our patient
3 years and 5 months after operation, and progress can be noted through the improvement of
hepatic function. The histopathological ﬁndings
showed hyperplastic adipose tissue consisting of
normal matured fat cells, which are peculiar forthis disease. Many patients without capsules havebeen reported, thus the capsule is not necessarily
speciﬁc to this lesion. It is also difﬁcult to distinguish with usual adipose tissue (Enzi et al., 1983).
In our patient, the lesion was inﬁltrative lipomatosis without capsules.
In this disease, surgery is the 1st choice oftreatment. An operation can be especially helpful
for patients with considerable cosmetic deformity
or presentation of a compressed airway. We decreased the volume of adipose tissue as much as
possible for our patient, and he was satisﬁed withhis appearance. Another choice of treatment has
been reported such as liposuction (Samdal et al.,References
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Received October 20, 2005; accepted November 14, 2005
Corresponding author: Yoshiyuki Tanji106