AJR 2002; 178:311-318
© American Roentgen Ray Society
Lipoatrophic—Lipodystrophic Syndromes
The Spectrum of Findings on MR Imaging
A. Premkumar1,
C. Chow1,
P. Bhandarkar1,
V. Wright1,
N. Koshy1,
S. Taylor2 and
E. Arioglu2
1
Department of Diagnostic Radiology, Warren Grant Magnuson Clinical Center,
National Institutes of Health, Bldg. 10, Rm. 1C660, 10 Center Dr. MSC 1182,
Bethesda, MD 20892-1182.
2
National Institute of Diabetes, Digestive and Kidney Diseases, National
Institutes of Health, Bldg. 10, Rm. 9S213 10, Center Dr. MSC 1829, Bethesda,
MD 20892-1829.
Received April 30, 2001;
accepted after revision July 10, 2001.
Presented at the annual meeting of the American Roentgen Ray Society,
Seattle, April 2001.
Address correspondence to A. Premkumar.
Introduction
Lipoatrophic—lipodystrophic syndromes comprise a heterogeneous group
of disorders associated with insulin-resistant diabetes and
hypertriglyceridemia [1].
Unlike the more common form of diabetes that is associated with obesity, the
lipoatrophic—lipodystrophic syndromes all cause either a lack of or an
abnormal distribution of adipose tissue. The similar metabolic abnormalities
in diabetes and lipoatrophic—lipodystrophic syndromes (i.e., obesity and
lipoatrophy) underscore the importance of adipose tissue in energy
homeostasis. In lipoatrophic—lipodystrophic syndromes, hypertension,
polycystic ovary syndrome, and acanthosis nigrans can be present in addition
to the insulin-resistant diabetes; these conditions are believed to be caused
by insulin resistance and hyperinsulinemia. Hepatic steatosis—which
sometimes progresses to cirrhosis—is uniformly seen in patients with the
severe forms of lipoatrophic and lipodystrophic syndromes. The loss of fat
produced by lipoatrophic disorders can be partial or generalized; it can be
acquired or congenital. Some correlation exists between the severity of
metabolic changes and the degree of fat
loss.
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Fig. 2D. —Familial partial lipodystrophy, or Dunnigan-Kobberling
syndrome, in 26-year-old woman. Axial MR imaging section obtained through
thighs shows no subcutaneous fat and minimal intra- and intermuscular fat. Fat
in bone marrow is preserved.
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The clinical diagnosis of lipodystrophic syndromes is based on the
presentation of the entity, physical appearance of the patient (exhibiting
characteristic distribution of fat), and metabolic abnormalities, including a
fasting insulin level of more than 30 µU/mL, fasting triglycerides level of
more than 200 mg/dL, and presence of diabetes as defined by the American
Diabetes Association criteria. In addition, gene mutations have been
identified for one form of the lipodystrophic disease, familial partial
lipodystrophy. At our institution, we have developed several criteria for the
diagnosis of the well-characterized genetic syndromes, which are listed in
Appendix 1.
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Fig. 4E. —Acquired general lipodystrophy, or Lawrence syndrome, in
young girl. Axial T1-weighted MR image (400/9; number of exitations, 2;
matrix, 256x192) obtained through thighs shows absence of fat in
subcutaneous and intramuscular regions but preservation of fat in bone
marrow.
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Because of its inherent ability to allow various tissue structures to be
differentiated from one another (particularly the differentiation of fat from
other types of soft tissue), cross-sectional MR imaging is ideally suited to
revealing lipoatrophic and lipodystrophic syndromes
[2].
Genetic Syndromes of Lipoatrophy
Although several genetic forms of lipoatrophy exist, two main syndromes are
most often recognized.
Congenital Generalized Lipoatrophy (Seip-Berardinelli Syndrome)
With an autosomal recessive pattern of inheritance, congenital generalized
lipoatrophy is a rare disease that has been found in all ethnic groups
[3]; fewer than 150 cases have
been reported in the literature. Our institution is a major referral center
for the evaluation and treatment of this disease; on the basis of the number
of probands that we have seen, we estimate that this disorder currently
affects approximately one in every five—10 million people in the United
States. Boys and girls are affected at similar rates, but the metabolic
features tend to be more severe and to develop earlier in girls. Patients
present within their first year of life with generalized absence of fat. They
develop insulin resistance, acanthosis nigricans, and diabetes mellitus by
adolescence. Gastrointestinal, neurologic, cardiac, and renal abnormalities
have been described in the literature. Hepatomegaly with cirrhosis is seen as
is accelerated growth with advanced skeletal maturation. Diffuse sclerosis of
the bony skeleton with focal lytic and sclerotic lesions have also been
reported.
MR images of the abdomen of patients with congenital generalized
lipoatrophy (Figs. 1A and
1B) show a complete absence of
intraabdominal, retroperitoneal, and subcutaneous fat. A fatty liver is
present, and the imaging sections obtained through the thighs reveal no
subcutaneous, intramuscular, or bone marrow fat
(Fig. 1C). However, the
presence of fat has been reported in some anatomic sites such as the orbits,
palms, and soles of the feet and in the periarticular regions. One hypothesis
for this finding is that the genetic defect in congenital generalized
lipoatrophy results in poor growth and development of metabolically active
adipose tissue, but mechanical adipose tissue deposition is preserved.
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Fig. 1A. —Congenital generalized lipoatrophy in 17 year-old boy.
(Imaging parameters for A—C are TR/TE, 400/9; number of
excitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section
obtained through upper abdomen shows increased signal intensity of liver due
to fatty infiltration.
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Fig. 1B. —Congenital generalized lipoatrophy in 17 year-old boy.
(Imaging parameters for A—C are TR/TE, 400/9; number of
excitations, 2; matrix, 256x192.) Axial MR imaging section obtained
through mid abdomen shows complete absence of subcutaneous and intraabdominal
fat.
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Fig. 1C. —Congenital generalized lipoatrophy in 17 year-old boy.
(Imaging parameters for A—C are TR/TE, 400/9; number of
excitations, 2; matrix, 256x192.) Axial MR imaging section obtained
through mid thigh shows no subcutaneous, intramuscular, or bone marrow
fat.
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Familial Partial Lipodystrophy (Dunnigan-Kobberling Syndrome)
Familial partial lipodystrophy is inherited in an autosomal dominant
fashion and has been found mostly in those of northern European descent,
although pedigrees of Asian descent have also been described. In certain
areas, such as rural Pennsylvania, and New Brunswick, Canada, the prevalence
of this disease may be as high as one or two of every 10,000 people, a finding
attributable to a founder effect from original German settlers. Probably
underdiagnosed in North America, familial partial lipodystrophy can often be
missed in male patients because, although both males and females are affected,
the disease is easier to discern in females.
Patients have normal fat distribution at birth but gradually lose
subcutaneous tissue in the extremities and trunk by early puberty. The loss of
fat in these areas is accompanied by normal or increased fat deposition in the
face and neck (Fig. 2A). Female
patients also develop more severe diabetes and hypertriglyceridemia than male
patients. Hepatic and renal diseases are rare in patients with this entity,
but polycystic ovaries are often seen
[4,
5]. This disease is associated
with mutations in a gene located on chromosome 19 encoding a nuclear envelope
protein called lamin A.
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Fig. 2A. —Familial partial lipodystrophy, or Dunnigan-Kobberling
syndrome, in 26-year-old woman. Photographs show increased adipose tissue in
patient's face and neck (A) and decreased fat in gluteal region and
lower extremities (B).
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MR images of the abdomen in patients with familial partial lipodystrophy
reveal a loss of subcutaneous fat and an increase in intraabdominal fat
(Fig. 2B). Imaging sections
obtained through the thighs reveal the loss of subcutaneous fat with the
preservation of or an increase in intramuscular, interfascial, and bone marrow
fat (Fig. 2C). The presentation
can be confused clinically with Cushing's syndrome; however, the absence of
subcutaneous fat and well-preserved muscles in the extremities enable one to
reach the correct diagnosis.
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Fig. 2B. —Familial partial lipodystrophy, or Dunnigan-Kobberling
syndrome, in 26-year-old woman. Photographs show increased adipose tissue in
patient's face and neck (A) and decreased fat in gluteal region and
lower extremities (B).
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Fig. 2C. —Familial partial lipodystrophy, or Dunnigan-Kobberling
syndrome, in 26-year-old woman. Axial T1-weighted MR image (TR/TE, 400/9;
number of exitations, 2; matrix, 256x192) of mid abdomen shows no
subcutaneous fat and increase in intraabdominal fat.
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New Familial Partial Lipodystrophy Syndrome
We have encountered an unusual syndrome in one family pedigree. This
autosomal dominant form of partial congenital lipodystrophy differs from
Dunnigan-Kobberling syndrome in that patients have an absence of fat in the
face, upper trunk, and arms as well as hypertrophic muscles and veins. An
associated progressive neurodegenerative disorder presents after puberty.
Congenital cataracts may be seen. Metabolic abnormalities include insulin
resistance, evidenced by high circulating insulin levels (>20 µU/mL)
associated with impaired glucose tolerance or diabetes (as defined by the
American Diabetes Association criteria) and elevated triglycerides.
MR images of the abdomen of patients with this disorder show some loss of
subcutaneous tissue with increased intrabdominal fat
(Fig. 3A). Unlike the features
seen in Dunnigan-Kobberling syndrome, increased subcutaneous fat is present in
the thighs (Fig. 3B). We are in
the process of reporting this syndrome in an effort to identify more
cases.
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Fig. 3A. —New familial partial lipodystrophy syndrome in 30-year-old
woman. (Imaging parameters for A and B are TR/TE, 400/9; number
of exitations, 2; matrix, 256x192.) Axial T1-weighted MR image of mid
abdomen shows decrease in subcutaneous adipose tissue and relative increase in
intraabdominal adipose tissue.
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Fig. 3B. —New familial partial lipodystrophy syndrome in 30-year-old
woman. (Imaging parameters for A and B are TR/TE, 400/9; number
of exitations, 2; matrix, 256x192.) Axial T1-weighted MR image reveals
increased subcutaneous fat in thighs.
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Acquired Syndromes of Lipoatrophy
Categorization of acquired forms of lipoatrophy can be difficult because of
the wide variation in presentation. Although there are various categories of
this entity, two specific eponyms are used in alluding to acquired
lipodystrophy—Lawrence-Seip syndrome and Barraquer-Simons' syndrome.
Acquired Generalized Lipodystrophy (Lawrence-Seip Syndrome)
The exact pathogenesis of acquired generalized lipodystrophy is unknown.
The findings of the original studies implicated a preceding chronic infection,
which is suggestive of an immunologic disturbance. The onset of the disease
occurs in childhood or early adolescence, and it is three times more common in
girls than in boys. Fat loss is severe, leading to a dramatic change in
physical appearance (Fig. 4A).
The clinical features are similar to congenital generalized lipoatrophy. After
the loss of fat, the median time for a patient to develop diabetes is 4 years.
A fatty liver is often seen, and the condition frequently progresses to
cirrhosis. Proteinuria and nephrotic syndrome are also seen
[3].
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Fig. 4A. —Acquired general lipodystrophy, or Lawrence syndrome, in
young girl. Photographs show patient before (A, taken at 3 years 8
months) and after (B, taken at 6 years 10 months) loss of fat that was
preceded by painful febrile panniculitis. Generalized fat loss developed in 16
weeks.
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MR images of patients with severe cases of acquired generalized
lipodystrophy reveal a general paucity of fat in subcutaneous and
intraabdominal sites (Figs. 4B
and 4C). A fatty liver can be
seen, along with a loss of subcutaneous and intramuscular fat in the
extremities (Fig. 4D). Bone
marrow fat may be lost in patients with more severe cases of the disorder.
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Fig. 4B. —Acquired general lipodystrophy, or Lawrence syndrome, in
young girl. Photographs show patient before (A, taken at 3 years 8
months) and after (B, taken at 6 years 10 months) loss of fat that was
preceded by painful febrile panniculitis. Generalized fat loss developed in 16
weeks.
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Fig. 4C. —Acquired general lipodystrophy, or Lawrence syndrome, in
young girl. Axial T1-weighted MR imaging sections (TR/TE, 400/9; number of
exitations, 2; matrix, 256x192) obtained through abdomen reveal fatty
liver (C) and loss of subcutaneous and intraabdominal fat
(D).
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Fig. 4D. —Acquired general lipodystrophy, or Lawrence syndrome, in
young girl. Axial T1-weighted MR imaging sections (TR/TE, 400/9; number of
exitations, 2; matrix, 256x192) obtained through abdomen reveal fatty
liver (C) and loss of subcutaneous and intraabdominal fat
(D).
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Acquired Partial Dystrophy (Barraquer-Simons' Syndrome)
Acquired partial dystrophy is the most common form of the lipodystrophies
and also one in which there is a wide range of variations
[6]. Patients usually are
females in the second or third decades of life. They often have a history of
an autoimmune disorder and present with partial loss of fat. The fat loss
starts in the face and upper half of the body and descends towards the gluteal
line. Often fat deposition increases in unaffected areas. Diabetes and
hypertriglyceridemia are seen in 50% of patients with acquired partial
dystrophy. Proteinuria and nephrotic syndrome are found more frequently than
in other forms of lipodystrophy.
MR images of patients with acquired partial dystrophy reveal a loss of
subcutaneous fat in the upper torso and in the anterior lower torso
(Fig. 5A). Increased
subcutaneous fat is seen posteriorly in the pelvis
(Fig. 5B) as well as in the
thighs (Fig. 5C).
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Fig. 5A. —Acquired partial dystrophy, or Barraquer-Simons' syndrome, in
60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9;
number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging
section of mid abdomen reveals loss of subcutaneous fat and relative increase
in intraabdominal fat.
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Fig. 5B. —Acquired partial dystrophy, or Barraquer-Simons' syndrome, in
60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9;
number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging
section through pelvis shows increased subcutaneous fat posteriorly with loss
of fat anteriorly.
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Fig. 5C. —Acquired partial dystrophy, or Barraquer-Simons' syndrome, in
60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9;
number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging
section reveals increased subcutaneous fat is also present in thighs.
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In a subset of patients with acquired partial dystrophy, fat distribution
does not follow the pattern seen in Barraquer-Simons' syndrome. In these
patients, MR images show a loss of subcutaneous fat with a relative increase
in intrabdominal fat (Fig.
6A), minimal subcutaneous fat, and minimal or no intramuscular
adipose tissue (Figs. 6B and
6C).
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Fig. 6A. —Acquired partial lipodystrophy in 56-year-old man. (Imaging
parameters of A and B are TR/TE, 400/9; number of exitations, 2;
matrix, 256x192.) Axial T1-weighted MR imaging section obtained through
abdomen shows loss of subcutaneous fat and increased intraabdominal fat.
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Fig. 6B. —Acquired partial lipodystrophy in 56-year-old man. (Imaging
parameters of A and B are TR/TE, 400/9; number of exitations, 2;
matrix, 256x192.) Axial T1-weighted MR imaging section obtained through
mid thigh reveals minimal subcutaneous fat and minimal (B) or no
(C) intramuscular fat.
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Fig. 6C. —Acquired partial lipodystrophy in 56-year-old man. (Imaging
parameters of A and B are TR/TE, 400/9; number of exitations, 2;
matrix, 256x192.) Axial T1-weighted MR imaging section obtained through
mid thigh reveals minimal subcutaneous fat and minimal (B) or no
(C) intramuscular fat.
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Dermatomyositis-Associated Lipodystrophy
A clear association exists between lipodystrophy and juvenile
dermatomyositis [7]. This form
of lipodystrophy is believed to be autoimmune-related, and patients typically
present before puberty. The lipodystrophy in these patients can progress from
a partial to a generalized form. Intraabdominal fat is often preserved despite
considerable loss of peripheral fat (Fig.
7A).
MR images of patients with dermatomyositis-associated lipodystrophy reveal
a loss of subcutaneous fat and an increase in intrabdominal fat. An asymmetry
of distribution of subcutaneous fat (Fig.
7B) is seen in the thighs, with more adipose tissue noted
anteromedially (Fig. 7C).
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Fig. 7B. —Dermatomyositis-associated lipodystrophy in 19-year-old
woman. Axial T1-weighted MR image (TR/TE, 400/9; number of exitations, 2;
matrix, 256 x 192) of abdomen shows no subcutaneous fat and increased
intraabdominal fat.
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Fig. 7C. —Dermatomyositis-associated lipodystrophy in 19-year-old
woman. Axial T1-weighted MR imaging section (400/9; number of exitations, 2;
matrix, 256 x 192) obtained through thighs shows asymmetrical
distribution of subcutaneous fat, with more fat deposited anteromedially.
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Other Causes of Acquired Lipodystrophy
Patients infected with HIV who are undergoing agrressive treatment can
develop partial lipoatrophy, particularly if they are taking protease
inhibitors [8]. The
lipodystrophy in these patients is characterized by a loss of subcutaneous fat
in the face, trunk, and extremities; an increase in intraabdominal fat; and,
in some patients, the development of a "buffalo hump" as seen in
patients with Cushing's syndrome. There is associated insulin resistance and
hypertriglyceridemia.
Lipodystrophy of Unknown Origin
Among the patients referred to our institution for lipodystrophic
syndromes, we found a group of patients—both males and females—who
fit into neither the genetic nor the acquired group. They present with
lipodystrophy in late childhood or as adults with the findings that are
suggestive of an autoimmune disease, although no apparent autoimmune disorders
are present.
The distribution of fat revealed on MR images of patients with this
disorder is also variable. Generally, more subcutaneous than intraabdominal
fat is found (Fig. 8A) as is
relatively less gluteal fat and "marbling" of the gluteus muscle
(Fig. 8B). The thighs show a
decrease of subcutaneous fat with some asymmetry in distribution of the fat.
Intramuscular adipose tissue is either minimal or absent
(Fig. 8C).
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Fig. 8A. —Lipodystrophy of unknown origin in 57-year-old man. (Imaging
Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix,
256 x 192.) Axial MR image shows increase in subcutaneous fat relative
to intraabdominal fat.
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Fig. 8B. —Lipodystrophy of unknown origin in 57-year-old man. (Imaging
Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix,
256 x 192.) Axial MR image reveals little gluteal fat and
"marbling" of gluteus muscle.
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Fig. 8C. —Lipodystrophy of unknown origin in 57-year-old man. (Imaging
Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix,
256 x 192.) Axial MR image reveals decrease in subcutaneous fat in
thighs, with slight asymmetry in distribution of fat.
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In conclusion, the ability of MR imaging to characterize the properties of
tissue makes it an excellent modality with which to reveal the spectrum of
findings in patients with lipoatrophic—lipodystrophic syndromes. This
diagnosis should be considered in diabetic patients whose imaging studies
reveal de novo distribution of fat typically seen in various forms of the
syndromes—especially in patients who also have laboratory findings that
are associated with the one of the many forms of the disorders. This knowledge
may help in evaluating patient response to therapy.
APPENDIX 1: Proposed Criteria for the Diagnosis of Well-Characterized
Genetic Syndromes of Lipoatrophy
Congenital Generalized Lipoatrophy (Presence of two of the
three major criteria and at least three supportive criteria needed for
diagnosis)
Major Criteria
- Autosomal recessive inheritance
- Paucity of fat apparent at birth or within the first year of life
- Emergence of at least one of the following metabolic abnormalities within
the first decade of life:
- Fasting insulin levels of more than 30mU/mL
- Fasting triglycerides levels of 200 mg/dL
- Presence of diabetes as defined by American Diabetes Association criteria
(fasting blood sugar > 126 mg/dL on two consecutive tests or 2-hr oral
glucose tolerance test glucose level > 200 mg/dL on two consecutive
tests)
- Enlarged liver with evidence of fatty infiltration and no other genetic
disease present
Supporting Criteria
- Acromegalic features
- Cardiomegaly
- Increased body hair during childhood
- Evidence of hyperandrogenism in girls
- Preservation of supportive fat in temporal fossa, palms, and soles of the
feet; presence of glandular breast tissue in girls
- Evidence of hypogonadotrophic hypogonadism
- Long bones with multiple sclerotic and lytic lesions
- MR images that reveal complete absence of fat in abdomen and extremities as
well as absence of bone marrow fat
- Early heavy proteinuria with no other features of nephrotic syndrome
- Leptin levels of less than 2 ng/mL
- Decreased IQ or attention deficit, particularly in boys
Familial Partial Lipodystrophy or Dunnigan-Kobberling Syndrome
(Presence of two major criteria or of one major and two supporting criteria
needed for diagnosis)
Major Criteria
- Autosomal dominant inheritance in pedigree (male patients are easy to miss;
therefore, at least one affected first-degree female relative required to
substantiate the diagnosis)
- Change in body habitus at or after puberty (clear increase of fat deposits
around face and neck)
- Presence of mutations on lamin A gene (if the test is available)
- Clear absence of subcutaneous fat in the extremities and trunk with
increased fat around face and neck or viscera (suspected on basis of physical
examination and supported by MR imaging findings)
At least one of the following metabolic abnormalities:
- Fasting insulin level of more than 30 mU/mL
- Fasting triglycerides level of more than 200 mg/mL
- Presence of diabetes as defined by American Diabetes Association
criteria
- Evidence of fatty infiltration of the liver
Supporting Criteria
- Presence of "buffalo hump"
- High-density lipoprotein level of less than 35 mg/dL
- Evidence of premature coronary artery disease
- Evidence of hyperandrogenism or menstrual abnormalities in female
patients
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Introduction
Genetic Syndromes of Lipoatrophy
