Diagnostic Considerations

Also consider the following:

Generalized lipodystrophies
Partial lipodystrophies
Facial hemiatrophy ^[25]
Panniculitis
Connective-tissue disorders associated with panniculitis
Lipodystrophy in HIV infection ^[26]
Lipoatrophic panniculitis ^[27]

Nakajo-Nishimura syndrome (ORPHA2615; also registered as Nakajo syndrome in OMIM#256040) is a distinct inherited inflammatory and wasting disease that usually begins in early infancy with a perniolike rash.^[28]Patients develop periodic high fever and nodular erythemalike eruptions, leading to gradually progress lipomuscular atrophy in the upper body, mainly the face and the upper extremities.

Focal congenital lipoatrophy was noted in a 2-year-old with a vascular malformation, with the suggestion that this child might have a mild form of inverse Klippel-Trenaunay syndrome.^[29]

The rare form of acquired partial lipodystrophy known as Barraquer-Simons syndrome may be associated with localized scleroderma.^[30]

Lipodystrophy may be stigmatizing, implying the use of highly active antiretroviral therapy (HAART) for HIV disease.^[31]

Linear lipoatrophy after intra-articular triamcinolone acetonide injection may mimic linear scleroderma.^[32]

Differential Diagnoses

Workup

Tandon VR, Gupte N, Mahajan V, Sharma R, Langer C, Khajuria V, et al. Bleomycin Containing Chemotherapeutic Regimen Induced Acquired Partial Lipodystrophy. Indian J Dermatol. 2016 Jan-Feb. 61 (1):122. [QxMD MEDLINE Link].
Payapvipapong K, Niumpradit N, Nakakes A, Buranawuti K. A rare case of acquired partial lipodystrophy (Barraquer-Simons syndrome) with localized scleroderma. Int J Dermatol. 2014 Jan. 53(1):82-4. [QxMD MEDLINE Link].
Capanni C, Mattioli E, Columbaro M, Lucarelli E, Parnaik VK, Novelli G, et al. Altered pre-lamin A processing is a common mechanism leading to lipodystrophy. Hum Mol Genet. 2005 Jun 1. 14(11):1489-502. [QxMD MEDLINE Link].
Atlan-Gepner C, Bongrand P, Farnarier C, Xerri L, Choux R, Gauthier JF, et al. Insulin-induced lipoatrophy in type I diabetes. A possible tumor necrosis factor-alpha-mediated dedifferentiation of adipocytes. Diabetes Care. 1996 Nov. 19(11):1283-5. [QxMD MEDLINE Link].
Reeves WG, Allen BR, Tattersall RB. Insulin-induced lipoatrophy: evidence for an immune pathogenesis. Br Med J. 1980 Jun 21. 280(6230):1500-3. [QxMD MEDLINE Link].
Monajemi H, Zhang L, Li G, Jeninga EH, Cao H, Maas M, et al. Familial partial lipodystrophy phenotype resulting from a single-base mutation in deoxyribonucleic acid-binding domain of peroxisome proliferator-activated receptor-gamma. J Clin Endocrinol Metab. 2007 May. 92(5):1606-12. [QxMD MEDLINE Link].
Demir T, Onay H, Savage DB, Temeloglu E, Uzum AK, Kadioglu P, et al. Familial partial lipodystrophy linked to a novel peroxisome proliferator activator receptor -γ (PPARG) mutation, H449L: a comparison of people with this mutation and those with classic codon 482 Lamin A/C (LMNA) mutations. Diabet Med. 2016 Jan 12. [QxMD MEDLINE Link].
Jeninga EH, van Beekum O, van Dijk AD, Hamers N, Hendriks-Stegeman BI, Bonvin AM, et al. Impaired Peroxisome Proliferator-Activated Receptor {gamma} Function through Mutation of a Conserved Salt Bridge (R425C) in Familial Partial Lipodystrophy. Mol Endocrinol. 2007 May. 21(5):1049-65. [QxMD MEDLINE Link].
Akinci B, Onay H, Demir T, Savas-Erdeve Ş, Gen R, Simsir IY, et al. Clinical presentations, metabolic abnormalities and end-organ complications in patients with familial partial lipodystrophy. Metabolism. 2017 Jul. 72:109-119. [QxMD MEDLINE Link].
Kim M, Oh Y, Hong SP, Jeon SY, Lee WS. Does segmental lipodystrophy represent mosaicism of inherited lipodystrophy?. J Am Acad Dermatol. 2009 Mar. 60(3):519-21. [QxMD MEDLINE Link].
Corredera C, Iglesias M, Hernández-Martín A, Colmenero I, Dilme E, Torrelo A. Annular lipoatrophic panniculitis of the ankles. Pediatr Dermatol. 2011 Mar. 28(2):146-8. [QxMD MEDLINE Link].
Gruber PC, Fuller LC. Lipoatrophy semicircularis induced by trauma. Clin Exp Dermatol. 2001 May. 26(3):269-71. [QxMD MEDLINE Link].
Breznik V, Kokol R, Luzar B, Miljkovic J. Insulin-induced localized lipoatrophy. Acta Dermatovenerol Alp Pannonica Adriat. 2013 Dec. 22(4):83-5. [QxMD MEDLINE Link].
Kayikcioglu A, Akyurek M, Erk Y. Semicircular lipoatrophy after intragluteal injection of benzathine penicillin. J Pediatr. 1996 Jul. 129(1):166-7. [QxMD MEDLINE Link].
Kumar V, Kumar M, Grover C. Localized lipoatrophy after intramuscular amikacin. Indian J Dermatol Venereol Leprol. 2009 Sep-Oct. 75(5):552. [QxMD MEDLINE Link].
Perez A, Nebot M, Macia M, Panades R. An outbreak of 400 cases of lipoatrophia semicircularis in Barcelona: effectiveness of control measures. J Occup Environ Med. 2010 Jul. 52(7):751-7. [QxMD MEDLINE Link].
Dahl PR, Zalla MJ, Winkelmann RK. Localized involutional lipoatrophy: a clinicopathologic study of 16 patients. J Am Acad Dermatol. 1996 Oct. 35(4):523-8. [QxMD MEDLINE Link].
Aviles-Izquierdo JA, Longo-Imedio MI, Hernanz-Hermosa JM, Lazaro-Ochaita P. Bilateral localized lipoatrophy secondary to a single intramuscular corticosteroid injection. Dermatol Online J. 2006 Mar 30. 12(3):17. [QxMD MEDLINE Link].
Ball NJ, Cowan BJ, Moore GR, Hashimoto SA. Lobular panniculitis at the site of glatiramer acetate injections for the treatment of relapsing-remitting multiple sclerosis. A report of two cases. J Cutan Pathol. 2008 Apr. 35(4):407-10. [QxMD MEDLINE Link].
Abbas O, Salman S, Kibbi AG, Chedraoui A, Ghosn S. Localized involutional lipoatrophy with epidermal and dermal changes. J Am Acad Dermatol. 2008 Mar. 58(3):490-3. [QxMD MEDLINE Link].
Imamura S, Yamada M, Ikeda T. Lipodystrophia centrifugalis abdominalis infantilis. Arch Dermatol. 1971 Sep. 104(3):291-8. [QxMD MEDLINE Link].
Imamura S. Lipodystrophia Centrifugalis Abdominalis Infantilis: Statistical Analysis of 168 Cases. Pediatr Dermatol. 2011 Dec 30. [QxMD MEDLINE Link].
Winkelmann RK, McEvoy MT, Peters MS. Lipophagic panniculitis of childhood. J Am Acad Dermatol. 1989 Nov. 21(5 Pt 1):971-8. [QxMD MEDLINE Link].
Commens C, O'Neill P, Walker G. Dermatomyositis associated with multifocal lipoatrophy. J Am Acad Dermatol. 1990 May. 22(5 Pt 2):966-9. [QxMD MEDLINE Link].
Hagari Y, Sasaoka R, Nishiura S, Ishihara M, Mihara M, Shimao S. Centrifugal lipodystrophy of the face mimicking progressive lipodystrophy. Br J Dermatol. 1992 Oct. 127(4):407-10. [QxMD MEDLINE Link].
Abrahams Z, Maartens G, Levitt N, Dave J. Anthropometric definitions for antiretroviral-associated lipodystrophy derived from a longitudinal South African cohort with serial dual-energy X-ray absorptiometry measurements. Int J STD AIDS. 2018 Jan 1. 956462418778649. [QxMD MEDLINE Link].
Comstock JR, Buhalog B, Peebles JK, Hinshaw MA, Co DO, Arkin LM. Lipoatrophic panniculitis in an adolescent. Pediatr Dermatol. 2020 Mar 20. [QxMD MEDLINE Link].
Kanazawa N. Nakajo-nishimura syndrome: an autoinflammatory disorder showing pernio-like rashes and progressive partial lipodystrophy. Allergol Int. 2012 Jun. 61(2):197-206. [QxMD MEDLINE Link].
Cappuccio G, Brunetti-Pierri N. Focal congenital lipoatrophy and vascular malformation: a mild form of inverse Klippel-Trenaunay syndrome?. Eur J Med Genet. 2012 Dec. 55(12):705-7. [QxMD MEDLINE Link].
Payapvipapong K, Niumpradit N, Nakakes A, Buranawuti K. A rare case of acquired partial lipodystrophy (Barraquer-Simons syndrome) with localized scleroderma. Int J Dermatol. 2013 May 15. [QxMD MEDLINE Link].
Kumar NS, Shashibhushan J, Malappa, Venugopal K, Vishwanatha H, Menon M. Lipodystrophy in Human Immunodeficiency Virus (HIV) Patients on Highly Active Antiretroviral Therapy (HAART). J Clin Diagn Res. 2015 Jul. 9 (7):OC05-8. [QxMD MEDLINE Link].
Call JE, Mann JA, Linos KD, Perry A, Yost J. Linear lipoatrophy following intra-articular triamcinolone acetonide injection mimicking linear scleroderma. Pediatr Dermatol. 2019 Jan. 36 (1):e56-e57. [QxMD MEDLINE Link].
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Media Gallery

Localized lipoatrophy from a steroid injection. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/lipoatrophy.jpg).

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

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, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former 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: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; 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 these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

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.

Sections Dermatologic Manifestations of Localized Lipodystrophy
Overview
Presentation
DDx
Workup
Treatment
Medication
References

Dermatologic Manifestations of Localized Lipodystrophy Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

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