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Dermatologic Manifestations of Localized Lipodystrophy Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 21, 2016
 

History

Lipodystrophia centrifugalis is defined by the appearance of cutaneous lesions surrounded by a peripheral erythematous border before age 3 years. They may progress centrifugally for as long as 10 years and tend to regress thereafter. Children who are affected are healthy.

Involutional lipoatrophy is characterized by one or a few circumscribed depressions of the skin affecting any part of the body in persons of any age, with or without a history of previous trauma, pressure, or previous injections in the area.[12] Clinically, it usually appears as a solitary, asymptomatic, well-demarcated, atrophic depression and often involves areas of antecedent intramuscular or intra-articular injections.[13, 14, 15] Overlying hypopigmentation and atrophy with morphealike changes may be noted with histology studies.

Panniculitis precedes the onset of lipoatrophy in the inflammatory subsets but may be asymptomatic clinically.

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Physical

Lipodystrophia centrifugalis abdominalis infantilis, as originally described by Imamura et al[16] in 1971, presents as a round or oval depression in the skin, often in the groin or the axilla.[17] It is characterized by one or a few circumscribed depressions or indentations in the skin. The overlying skin is of normal texture and color except for the periphery, which may be erythematous and a little scaly. Lesions tend to spread centrifugally to adjacent areas, such as the chest and the abdomen, for several years. The face and the sacral area can also be involved. Regional enlarged lymph nodes can often be palpated. No other abnormal finding is present clinically.

The lesions are round, oval, bandlike, horizontal, annular, or semicircular in configuration. They are flesh colored and barely visible with normal overlying skin. The anterior parts of the thighs, the buttocks, or the lower parts of the limbs, including the ankles in some cases, as well as the arms, the neck, and the scalp may be involved, unilaterally or bilaterally. The lesions may be mildly tender at first. No associated finding and no preceding sign of inflammation are present.

Localized lipoatrophy may represent the late stage of panniculitis. Indurated, tender, erythematous, purplish or hyperpigmented subcutaneous nodules or plaques usually involving the extremities precede the onset of lipoatrophy at the same sites.

Fever and systemic signs may be present depending on the type of associated panniculitis.

Lipophagic panniculitis of childhood, first described in children and later in adults,[18] is characterized by its benign self-limited course. It presents with multiple, slightly tender, erythematous subcutaneous nodules and plaques, which precede the onset of lipoatrophy. The lesions mainly occur on the extremities. It may recur several times, and it may be associated with intermittent fever.

Localized lipoatrophy may be the only presenting feature in patients with an underlying or evolving connective-tissue disorder, such as lupus erythematosus, morphea profunda, dermatomyositis,[19] or overlap disease. In patients with lupus panniculitis, multiple, often painful subcutaneous nodules or plaques, with a predilection for the proximal extremities, may be observed in association with irregular, indurated depressions in the same areas. The overlying skin is often normal, or changes, such as atrophy, hyperkeratosis, telangiectasias, and/or ulceration, seen in discoid lupus erythematosus may be present. Arthralgias or Raynaud syndrome may be observed, but most patients show no evidence of severe systemic involvement, such as glomerulonephritis or neurologic complications.

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Causes

The cause of centrifugal lipodystrophy is unknown.

In many cases, involutional lipodystrophy is idiopathic. In other cases, repeated trauma,[20] chronic pressure or compression, or local injections may be the cause. It has been reported to develop at the site of injection or in the surrounding area of injection with insulin,[21] intralesional or intra-articular steroids, antibiotics (eg, intramuscular benzathine penicillin),[22] vasopressin, and human growth hormone.[21] Localized lipoatrophy was also noted following intramuscular injection of amikacin.[23]

Localized lipoatrophy may represent the late or end stage of a preceding or concomitant panniculitis, which may be triggered by various underlying conditions. Connective-tissue disease–induced panniculitis is one of the main causes and may not be symptomatic at the time of presentation. Other causes of lipoatrophic panniculitis may include alpha-1-antitrypsin deficiency or cytophagic, factitial, and infectious panniculitides.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Isabelle Thomas, MD Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange

Isabelle Thomas, MD is a member of the following medical societies: American Academy of Dermatology, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Daniel Mark Siegel, MD, MS Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate Medical Center

Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Association for Physician Leadership, American Society for Dermatologic Surgery, American Society for MOHS Surgery, International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

References
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