Dermatologic Manifestations of Localized Lipodystrophy Differential Diagnoses

Updated: Apr 16, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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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