Acquired generalized lipodystrophy - Revision history

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2025-04-03T22:20:42Z

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			{{Infobox medical condition
			\| name = Acquired generalized lipodystrophy
			\| synonyms = Lawrence syndrome
			\| field = [[Endocrinology]]
			\| symptoms = Loss of [[adipose tissue]], [[insulin resistance]], [[hypertriglyceridemia]], [[acanthosis nigricans]], [[hepatic steatosis]]
			\| complications = [[Diabetes mellitus]], [[pancreatitis]], [[cardiovascular disease]]
			\| onset = Childhood or adolescence
			\| duration = Chronic
			\| causes = Autoimmune, [[infections]], [[medications]]
			\| risks = [[Autoimmune diseases]], [[HIV infection]], certain [[medications]]
			\| diagnosis = [[Clinical evaluation]], [[imaging studies]], [[biopsy]]
			\| differential = [[Congenital generalized lipodystrophy]], [[partial lipodystrophy]], [[Cushing's syndrome]]
			\| treatment = [[Dietary management]], [[metformin]], [[insulin therapy]], [[leptin replacement therapy]]
			\| prognosis = Variable, depends on complications
			\| frequency = Rare
			}}
	== '''Alternate names''' ==		== '''Alternate names''' ==
	Lawrence syndrome; Lawrence-Seip syndrome; Acquired lipoatrophic diabetes		Lawrence syndrome; Lawrence-Seip syndrome; Acquired lipoatrophic diabetes

	== '''Definition''' ==		== '''Definition''' ==
	A rare lipodystrophic syndrome characterized by loss of [[adipose tissue]], and is a syndrome of [[insulin]] resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of [[subcutaneous]] adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles).		A rare lipodystrophic syndrome characterized by loss of [[adipose tissue]], and is a syndrome of [[insulin]] resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of [[subcutaneous]] adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles).

	== '''Epidemiology''' ==		== '''Epidemiology''' ==
	More than 100 cases have been described and the female to male ratio is 3:1.		More than 100 cases have been described and the female to male ratio is 3:1.

	== '''Cause''' ==		== '''Cause''' ==
	* The cause of the disease remains unknown.		* The cause of the disease remains unknown.
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	* Progression towards partial lipoatrophy, focal or generalized, has been reported in patients with [[dermatomyositis]], amongst whom this could be a late relapse, and it is more common that the antibody anti-p155 is present.		* Progression towards partial lipoatrophy, focal or generalized, has been reported in patients with [[dermatomyositis]], amongst whom this could be a late relapse, and it is more common that the antibody anti-p155 is present.
	* The hypothesis of an underlying genetic factor has not been rejected.		* The hypothesis of an underlying genetic factor has not been rejected.

	== '''Signs and symptoms''' ==		== '''Signs and symptoms''' ==
	* The clinical phenotype is similar to that of Berardinelli-Seip syndrome , but lipoatrophy appears secondarily during childhood, adolescence or adulthood, and as a result the syndrome is thought to be acquired.		* The clinical phenotype is similar to that of Berardinelli-Seip syndrome , but lipoatrophy appears secondarily during childhood, adolescence or adulthood, and as a result the syndrome is thought to be acquired.
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	* [[Proteinuria]] associated with focal segmental [[glomerulosclerosis]] or with [[membranoproliferative glomerulonephritis]] have been reported recently, as well as dysregulation of growth hormone.		* [[Proteinuria]] associated with focal segmental [[glomerulosclerosis]] or with [[membranoproliferative glomerulonephritis]] have been reported recently, as well as dysregulation of growth hormone.
	* Three types of the disease have been described: 1) a form with [[panniculitis]] (inflammatory nodules followed by lipoatrophy), 2) an autoimmune form that is readily associated with other syndromes such as chronic active [[hepatitis]], Hashimoto struma and [[hemolytic anemia]], but also with [[dermatomyositis]] and [[Sjogren's syndrome]] , 3) idiopathic.		* Three types of the disease have been described: 1) a form with [[panniculitis]] (inflammatory nodules followed by lipoatrophy), 2) an autoimmune form that is readily associated with other syndromes such as chronic active [[hepatitis]], Hashimoto struma and [[hemolytic anemia]], but also with [[dermatomyositis]] and [[Sjogren's syndrome]] , 3) idiopathic.

	== '''Clinical presentation''' ==		== '''Clinical presentation''' ==
	For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.		For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.
	'''100% of people have these symptoms'''		'''100% of people have these symptoms'''
	* Generalized [[lipodystrophy]]		* Generalized [[lipodystrophy]]

	'''80%-99% of people have these symptoms'''		'''80%-99% of people have these symptoms'''
	* [[Hyperinsulinemia]]		* [[Hyperinsulinemia]]

	'''30%-79% of people have these symptoms'''		'''30%-79% of people have these symptoms'''
	* Autoimmunity(Autoimmune disease)		* Autoimmunity(Autoimmune disease)
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	* Insulin-resistant diabetes mellitus(Insulin resistant diabetes)		* Insulin-resistant diabetes mellitus(Insulin resistant diabetes)
	* Progeroid facial appearance(Premature aged appearance)		* Progeroid facial appearance(Premature aged appearance)

	'''5%-29% of people have these symptoms'''		'''5%-29% of people have these symptoms'''
	* Abnormality of complement system		* Abnormality of complement system
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	* Polycystic ovaries		* Polycystic ovaries
	* [[Proteinuria]](High urine protein levels)		* [[Proteinuria]](High urine protein levels)

	'''1%-4% of people have these symptoms'''		'''1%-4% of people have these symptoms'''
	* [[Astrocytoma]]		* [[Astrocytoma]]
	* [[Lymphoma]](Cancer of lymphatic system)		* [[Lymphoma]](Cancer of lymphatic system)
	* Unicameral bone cyst		* Unicameral bone cyst

	== '''Diagnosis''' ==		== '''Diagnosis''' ==
	Diagnosis is clinical and should be confirmed by an assessment of body fat, in particular by biphotonic absorptiometry and [[magnetic resonance imaging]].		Diagnosis is clinical and should be confirmed by an assessment of body fat, in particular by biphotonic absorptiometry and [[magnetic resonance imaging]].

	== '''Treatment''' ==		== '''Treatment''' ==
	* The treatment of the metabolic manifestations is a priori no different to the treatment of other forms of insulin resistance: physical exercise, insulin sensitizers such as [[metformin]] or [[pioglitazone]], insulin (or preferably insulin analogues), [[antihypertensives]], and monitoring and treatment of [[hypertriglyceridemia]].		* The treatment of the metabolic manifestations is a priori no different to the treatment of other forms of insulin resistance: physical exercise, insulin sensitizers such as [[metformin]] or [[pioglitazone]], insulin (or preferably insulin analogues), [[antihypertensives]], and monitoring and treatment of [[hypertriglyceridemia]].
	* The efficacy of recombinant human [[leptin]] has been demonstrated on the metabolic level but this therapy is not available in all countries.		* The efficacy of recombinant human [[leptin]] has been demonstrated on the metabolic level but this therapy is not available in all countries.
	* In serious [[autoimmune]] forms of the disease, [[immunosuppressive therapy]] may be indicated.		* In serious [[autoimmune]] forms of the disease, [[immunosuppressive therapy]] may be indicated.

	== '''Prognosis''' ==		== '''Prognosis''' ==
	The prognosis is not well known but is probably related to cardiovascular risk (linked to the insulin-resistance syndrome) and to the underlying cause of the disease.		The prognosis is not well known but is probably related to cardiovascular risk (linked to the insulin-resistance syndrome) and to the underlying cause of the disease.

	{{Disorders of subcutaneous fat}}		{{Disorders of subcutaneous fat}}

	[[Category:Conditions of the subcutaneous fat]]		[[Category:Conditions of the subcutaneous fat]]
	[[Category:Syndromes]]		[[Category:Syndromes]]

Prab at 16:45, 22 March 2025

2025-03-22T16:45:45Z

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Deepika vegiraju at 14:08, 29 April 2021

2021-04-29T14:08:24Z

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== '''Alternate names''' ==
Lawrence syndrome; Lawrence-Seip syndrome; Acquired lipoatrophic diabetes

== '''Definition''' ==
A rare lipodystrophic syndrome characterized by loss of [[adipose tissue]], and is a syndrome of [[insulin]] resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of [[subcutaneous]] adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles).

== '''Epidemiology''' ==
More than 100 cases have been described and the female to male ratio is 3:1.

== '''Cause''' ==
* The cause of the disease remains unknown.
* There may be infectious triggering factors (there was a recent case of the [[panniculitis]] type that appeared after [[tuberculosis]]) or an [[autoimmune]] mechanism.
* A recent publication showed activation of the classical complement pathway (low C4). This is in contrast to [[acquired partial lipodystrophy]] which affects the upper half of the body and is characterized by an activation of the alternative complement pathway (low C3).
* Progression towards partial lipoatrophy, focal or generalized, has been reported in patients with [[dermatomyositis]], amongst whom this could be a late relapse, and it is more common that the antibody anti-p155 is present.
* The hypothesis of an underlying genetic factor has not been rejected.

== '''Signs and symptoms''' ==
* The clinical phenotype is similar to that of Berardinelli-Seip syndrome , but lipoatrophy appears secondarily during childhood, adolescence or adulthood, and as a result the syndrome is thought to be acquired.
* In some cases, loss of [[adipose tissue]] is localized, especially if it is preceded by a [[panniculitis]].
* The syndrome is associated with a [[voracious]] appetite and an acceleration of growth in adolescents.
* One third of cases are accompanied by [[acanthosis nigricans]] and a [[polycystic ovary syndrome]] .
* [[Hepatomegaly]] with [[steatosis]] and a risk of cirrhogenous progression is common.
* Biologically, [[hyperinsulinemia]] and insulin-resistant diabetes are observed, often associated with severe [[hypertriglyceridemia]] with low plasma levels of [[leptin]] and [[adiponectin]].
* [[Proteinuria]] associated with focal segmental [[glomerulosclerosis]] or with [[membranoproliferative glomerulonephritis]] have been reported recently, as well as dysregulation of growth hormone.
* Three types of the disease have been described: 1) a form with [[panniculitis]] (inflammatory nodules followed by lipoatrophy), 2) an autoimmune form that is readily associated with other syndromes such as chronic active [[hepatitis]], Hashimoto struma and [[hemolytic anemia]], but also with [[dermatomyositis]] and [[Sjogren's syndrome]] , 3) idiopathic.

== '''Clinical presentation''' ==
For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.
'''100% of people have these symptoms'''
* Generalized [[lipodystrophy]]

'''80%-99% of people have these symptoms'''
* [[Hyperinsulinemia]]

'''30%-79% of people have these symptoms'''
* Autoimmunity(Autoimmune disease)
* Calf muscle pseudohypertrophy
* [[Cardiomyopathy]](Disease of the heart muscle)
* Hepatic [[steatosis]](Fatty infiltration of liver)
* Insulin-resistant diabetes mellitus(Insulin resistant diabetes)
* Progeroid facial appearance(Premature aged appearance)

'''5%-29% of people have these symptoms'''
* Abnormality of complement system
* [[Acanthosis nigricans]](Darkened and thickened skin)
* Accelerated skeletal maturation(Advanced bone age)
* Acute [[pancreatitis]](Acute pancreatic inflammation)
* [[Cirrhosis]](Scar tissue replaces healthy tissue in the liver)
* Generalized hirsutism(Excessive hairiness over body)
* Generalized [[hyperpigmentation]]
* [[Hepatomegaly]](Enlarged liver)
* [[Hypertension]]
* Hypertriglyceridemia(Increased plasma triglycerides)
* [[Myopathy]](Muscle tissue disease)
* [[Panniculitis]](Inflammation of fat tissue)
* Polycystic ovaries
* [[Proteinuria]](High urine protein levels)

'''1%-4% of people have these symptoms'''
* [[Astrocytoma]]
* [[Lymphoma]](Cancer of lymphatic system)
* Unicameral bone cyst

== '''Diagnosis''' ==
Diagnosis is clinical and should be confirmed by an assessment of body fat, in particular by biphotonic absorptiometry and [[magnetic resonance imaging]].

== '''Treatment''' ==
* The treatment of the metabolic manifestations is a priori no different to the treatment of other forms of insulin resistance: physical exercise, insulin sensitizers such as [[metformin]] or [[pioglitazone]], insulin (or preferably insulin analogues), [[antihypertensives]], and monitoring and treatment of [[hypertriglyceridemia]].
* The efficacy of recombinant human [[leptin]] has been demonstrated on the metabolic level but this therapy is not available in all countries.
* In serious [[autoimmune]] forms of the disease, [[immunosuppressive therapy]] may be indicated.

== '''Prognosis''' ==
The prognosis is not well known but is probably related to cardiovascular risk (linked to the insulin-resistance syndrome) and to the underlying cause of the disease.

{{Disorders of subcutaneous fat}}

[[Category:Conditions of the subcutaneous fat]]
[[Category:Syndromes]]
{{rarediseases}}
{{stub}}