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Lipodystrophy or Lipoatrophy

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Prevalence
Semicircular Localized Lipodystrophy
Diagnosis
Causes
Autoimmune
Dermatomyositis
Electromagnetic Fields?
Gluten Sensitivity
Lupus
Panniculitis
Scleroderma
Sjogren's Syndrome
Thyroiditis
Treatments

Overview of Lipodystrophy or Lipoatrophy

Lipodystrophy (or lipoatrophy) refers to a loss of fat tissue. It can be localized, partial, or total.
Types of Lipodsytrophies. There are several different types of lipodystrophies and the degree of fat loss may vary from very small depressed areas to near complete absence of adipose tissue. Inherited lipodystrophies are caused by mutations in a gene. Acquired lipodystrophies are caused by medications, autoimmune mechanisms or other unknown mechanisms. Southwestern Medical Center.
This page covers only localized lipodystrophy that is caused by autoimmune diseases. Other causes include AIDS/HIV or retroviral treatments.

Prevalence of Localized Lipodystrophy

PatientCrossroads and Lipodystrophy United Launch Global Registry for Lipodystrophy. No one knows exactly how many people are affected by lipodystrophy worldwide, and clinical endpoints that could gauge the effectiveness of treatments are sparse. PR Web, 03/04/2014.
Localized Lipodystrophy. Because localized lipodystrophy is extremely rare, only a few case series of lipodystrophic syndromes are reported in the literature. Emedicine.

Semicircular Localized Lipodystrophy or Lipoatrophy

Semicircular Localized Lipodystrophy. Lipoatrophy can be a common sequela of panniculitis in patients with connective tissue diseases (eg, systemic lupus erythematosus, subcutaneous morphea, the syndrome of lobular lymphocytic connective tissue panniculitis of Winkelmann and Padilha-Goncalves). Lipoatrophy can be associated with nephritis, hypocomplementemia, scleroderma, Sjögren syndrome, recurrent pyogenic infections, immune thrombocytopenic purpura (ITP), and thyroiditis. Emedicine.
Lipoatrophia Semicircularis: a new office disease? 900 cases reported in Belgium. This phenomenon was reported for the first time in three patients in 1974 by Gschwandtner and Munzberger. Since then, there have been some publications, but these relate only to 70-odd patients. Since 1995, we have diagnosed more than 900 cases in our company. Although the cause is still unknown, we believe that electromagnetic fields play an important role in this phenomenon. Curvers Bart, MD, ESD Journal.

Diagnosis of Lipodystrophy

Lipodystrophy, Progressive: Differential Diagnoses & Workup. Urinalysis, baseline creatinine and blood urea nitrogen studies, as well as urine studies, a CBC count, and other tests are recommended for lipodystrophy. Robert A Schwartz, MD. eMedicine. June, 2010.

Causes of Lipodystrophy: Autoimmune Diseases

Lipoatrophy can be a common sequela of panniculitis in patients with connective tissue diseases (eg, systemic lupus erythematosus, subcutaneous morphea, the syndrome of lobular lymphocytic connective tissue panniculitis of Winkelmann and Padilha-Goncalves). Lipoatrophy can be associated with nephritis, hypocomplementemia, scleroderma, Sjögren's syndrome, recurrent pyogenic infections, immune thrombocytopenic purpura (ITP), and thyroiditis. eMedicine.
Lipodystrophy, Acquired Partial. Disorders associated with acquired partial lipodystrophy include membranoproliferative glomerulonephritis, and autoimmune diseases including (but not limited to) systemic lupus erythematosus, dermatomyositis, pernicious anemia, celiac disease, dermatitis herpetiformis, rheumatoid arthritis, temporal arteritis, leukocytoclastic vasculitis, POEMS, and diabetes. eMedicine.
Primary Atrophic Solitary Morphea Profunda. Here, we describe a patient presenting with noninflammatory cupuliform (shaped like a small cup) depressed plaques, without any significant skin induration, pigmentation or texture change, that appeared on the left shoulder at a site of previous intramuscular vaccination. Our observation highlights the wide spectrum of clinical presentations of localized scleroderma. The latter should be considered in the presence of lipoatrophy-like lesions for proper workup and therapy. (Also see: Causes of Scleroderma: Vaccinations and Morphea Profunda)

Causes of Lipodystrophy: Dermatomyositis

Lipodystrophy and metabolic abnormalities in a case of adult dermatomyositis. Loss of subcutaneous tissue may be a cutaneous marker for metabolic abnormalities in both the adult and the juvenile forms of dermatomyositis. J Am Acad Dermatol;57:S85-7.
Study of subcutaneous fat in children with juvenile dermatomyositis. Sixty-five percent of our patients with juvenile DM were found to have loss of subcutaneous fat on quantification compared with 40% on physical appearance alone. Lipodystrophy and hypertriglyceridemia could well be integral components of what may be an expanded juvenile DM syndrome. Arthritis Care and Research Journal, vol. 55, no4, pp. 564-568.

Causes of Lipodystrophy: Diabetes

Lipoatrophic diabetes mellitus. Lipoatrophic diabetes mellitus is characterized by a lack of subcutaneous fat (lipoatrophy), high blood sugar (hyperglycemia), and high blood insulin (hyperinsulinemia). SteadyHealth.com Encyclopedia.

Causes of Localized Lipodystrophy: Electromagnetic Fields?

Lipoatrophia Semicircularis: a new office disease? 900 cases reported in Belgium. Since 1995, we have diagnosed more than 900 cases in our company. Based on our research, we believe that electromagnetic fields play an important role in this phenomenon. Curvers Bart, MD, ESD Journal.

Causes of Lipodystrophy: Gluten Sensitivity

Dermatomyositis associated with celiac disease: response to a gluten-free diet. After she was put on a strict gluten-free diet, both nutritional deficiencies and the dermatomyositis resolved. The patient's human leukocyte antigen haplotype study was positive for DR3 and DQ2, which have been shown to be associated with both juvenile dermatomyositis and celiac disease. It is suggested that patients with newly diagnosed dermatomyositis be investigated for concomitant celiac disease even in the absence of gastrointestinal symptoms. Can J Gastroenterol. 2006 Jun;20(6):433-5.(Also see: Celiac Disease and Gluten Sensitivity)

Causes of Lipodystrophy: Lupus

Partial Lipodystrophy in a Patient with Systemic Lupus erythematosus. A 54-year-old woman developed partial lipodystrophy on the left side of her face. She had been suffering from systemic lupus erythematosus (SLE) since 1985 when she was 45 years old. Dermatology 2002;204:298-300.
Semicircular lipoatrophy in a child with systemic lupus erythematosus after subcutaneous injections with methotrexate. We report a 10-year-old girl who developed semicircular lipoatrophy on the anterior thighs after treatment of systemic lupus erythematosus (SLE) with subcutaneous injections of methotrexate. Pediatr Dermatol. 2002 Sep-Oct;19(5):432-5.

Causes of Lipodystrophy: Panniculitis

Panniculitis. Panniculitis is inflammation of the subcutaneous fat. Fat is divided into lobules by connective tissue septae. These septae contain the blood supply supplying the lobule. Chart lists type of infiltrates associated by diseases, including lipoatrophy. The Doctor's Doctor.
Connective tissue panniculitis: lupus panniculitis, dermatomyositis, morphea/scleroderma.These subtypes of panniculitis are unified by an active inflammatory stage of the disease that can progress to develop scarring, atrophy, and calcifications. CB Hansen (PubMed) Dermatol Ther. 2010 Jul-Aug;23(4):341-9.
Lipodystrophy and connective tissue panniculitis. The case describes an acquired lipodystrophy with accompanying diabetes mellitus and positive autoantibodies. This association without clinical features of connective tissue disease suggests that lipodystrophy is part of a clinical spectrum, rather than a distinct clinical entity. Rheumatology 2001; 40: 1070-1071.

Causes of Lipodystrophy: Scleroderma

Lipoatrophy and Scleroderma. Middle-age woman with a history of diffuse scleroderma for more than 15 years. The patient develops lipoatrophy of the face and calcinosis. DermAtlas.
Morphea/Scleroderma/Lipoatrophy (Photo). This healthy 16-year-old girl developed lipoatrophy underlying a plaque of linear morphea. Bernard Cohen, MD. DermAtlas.
Primary Atrophic Solitary Morphea Profunda (SMP) is a distinct form of localized scleroderma, mimicking localized lipoatrophy. Our observation highlights the wide spectrum of clinical presentations of localized scleroderma. The latter should be considered in the presence of lipoatrophy-like lesions for proper workup and therapy. Karger Dermatology.
Parry Romberg Syndrome with Involuntary Jaw Closure: An Extremely Rare Association. In lipodystrophy, the involvement is bilateral and spares cartilages and bone; in scleroderma the skin becomes cold, white and inelastic and adherent to the subcutaneous tissue. The skin in PRS remains mobile and grossly normal. JIACM 2006; 7(3): 258-60.
Sclerodermic linear lupus panniculitis: report of two cases. Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. PubMed. Dermatology. 2005;210(4):329-32. (Also see: Linear Scleroderma, and Overlap Syndrome)

Causes of Lipodystrophy: Sjogren's Syndrome

Lipoatrophy can be a common sequela of panniculitis in patients with connective tissue diseases (eg, systemic lupus erythematosus, subcutaneous morphea, the syndrome of lobular lymphocytic connective tissue panniculitis of Winkelmann and Padilha-Goncalves). Lipoatrophy can be associated with nephritis, hypocomplementemia, scleroderma, Sjögren's syndrome, recurrent pyogenic infections, immune thrombocytopenic purpura (ITP), and thyroiditis. Emedicine. (Also see: Sjogren's Syndrome)

Causes of Lipodystrophy: Thyroiditis

Lipoatrophy can be a common sequela of panniculitis in patients with connective tissue diseases (eg, systemic lupus erythematosus, subcutaneous morphea, the syndrome of lobular lymphocytic connective tissue panniculitis of Winkelmann and Padilha-Goncalves). Lipoatrophy can be associated with nephritis, hypocomplementemia, scleroderma, Sjögren's syndrome, recurrent pyogenic infections, immune thrombocytopenic purpura (ITP), and thyroiditis. Emedicine. (Also see: Thyroid Disease)

Treatments for Lipodystrophy

Rosiglitazone Abrogates Bleomycin-Induced Scleroderma and Blocks Profibrotic Responses Through Peroxisome Proliferator-Activated Receptor-{gamma}. Collectively, these results indicate that rosiglitazone treatment attenuates inflammation, dermal fibrosis, and subcutaneous lipoatrophy via PPAR-{gamma} in a mouse model of scleroderma and suggest that pharmacological PPAR-{gamma} ligands, widely used as insulin sensitizers in the treatment of type-2 diabetes mellitus, may be potential therapies for scleroderma. American Journal of Pathology. 2009;174:519-533.
Autologous fat grafting. Autologous fat grafting is a standard method for soft tissue augmentation. The method is commonly used for volume restoration of the aging face. Furthermore, atrophic scars, lipodystrophy and scleroderma en coup de sabre can be treated. Hautarzt. 2003 Dec;54(12):1185-9.
 
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