Lipodystrophies E88.10

Very differently used and therefore not clearly defined term for a localized or generalized, congenital or acquired, partial or complete loss of (subcutaneous) fatty tissue.

The term lipodystrophy is often used synonymously with lipoatrophy.

However, lipodystrophy includes lipatrophy.

Lipodystrophy can be accompanied by a simultaneous redistribution of fatty tissue (e.g. HIV-induced lipodystrophy).

A classification of lipodystrophies according to their mode of origin (acquired or congenital/familial), their pattern of affection (localized, partial, or generalized), and the presence of endocrinologic (diabetes mellitus) and other internal disorders (hepatomegaly, glomerulonephritis, hypertriglyceridemia) is accepted.

• Congenital/familial:
  • Generalized:
    • Lipodystrophy, generalized (congenital generalized lipatrophy).
  • Partial:
    • Familial partial lipodystrophy syndromes:
      • Koebberling-Dunningham type (type 1)
      • Dunningham type (type 2)
      • Barrosa type (type 3)
    • Lipodystrophy with mandibuloacral dysplasia

    • SHORT syndrome (mutation in PIK3R1 gene/171833/ on chromosome 5q13)

• Acquired:
  • Generalized:
    • Lipodystrophy, generalized, acquired (Lawrence syndrome).
  • Partial:
    • Lipodystrophycaused by protease inhibitors (HIV)
    • Lipodystrophy, progressive partial (Barraquer-Simons).
  • Localized:
    • Traumatic (e.g., after wearing lacing garments (e.g., bra straps, rings, belts).
    • Lipoatrophy semicircularis (anularis)
    • Lipoatrophy after injections (glucocorticoids, insulin, growth hormone)
    • Lipodystrophy after panniculitis
    • Lipodystrophy after/in collagenoses(morphea, lupus erythematosus profundus).
• Unexplained:
  • Lipodystrophia centrifugalis abdominalis infantilis
  • Panatrophia localisata (Gowers)
  • Hemiatrophia faciei progressiva (Parry Romberg)
  • Non-progressive late onset hemifacial lipoatrophy.

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