Table 1 Lipodystrophy subtypes (adapted from (2))

1 Congenital

 1.1 Generalized

  Type 1 CGL (AGPAT2, recessive, OMIM #608594)

  Type 2 CGL (BSCL2, recessive, OMIM #269700)

  Type 3 CGL (CAV1, recessive, OMIM #612526)

  Type 4 CGL (PTRF, recessive, OMIM #613327)

PPARG -associated CGL (recessive)

  Progressive Encephalopathy with/without lipodystrophy (BSCL2, recessive, OMIM: #615924)

 1.2 Partial

  Type 1 FPLD (Köbberling syndrome; genes unknown, OMIM %608,600)

  Type 2 FPLD (Dunnigan disease; LMNA, (co-)dominant, OMIM #151660)

  Type 3 FPLD (PPARG, dominant, OMIM #604367)

  Type 4 FPLD (PLIN1, dominant, OMIM #613877)

  Type 5 FPLD (CIDEC, recessive, OMIM #615238)

  Type 6 FPLD (LIPE, recessive, OMIM #615980)

  Type 7 FPLD with congenital cataracts, and neurodegeneration (CAV1, dominant, OMIM #606721)

AKT2-linked lipodystrophy (dominant)

MFN2 associated FPLD (recessive)

ADRA2A associated FPLD (dominant)

 1.3 Systemic

  1.3.1 Progeroid syndromes

   Hutchinson-Gilford progeria syndrome (LMNA, dominant, OMIM #176670)

   Néstor-Guillermo progeria syndrome (BANF1, recessive, OMIM #614008)

   Atypical Werner syndrome and atypical progeroid syndrome (de novo, LMNA-associated)

   Werner syndrome (RECQL2, recessive, OMIM #277700)

   Type A mandibuloacral dysplasia (LMNA, recessive, OMIM #248370)

   Type B mandibuloacral dysplasia (ZMPSTE24, recessive, OMIM #608612)

   SHORT syndrome (PIK3R1, dominant, OMIM #269880)

   MDPL syndrome (POLD1, dominant, OMIM #615381)

   Keppen-Lubinsky syndrome (KCNJ6, dominant, OMIM #614098)

   Ruijs-Aalfs syndrome (SPRTN, recessive, OMIM #616200)

   Cockayne syndrome (ERCC6,recessive, OMIM #133540)

   Cockayne syndrome (ERCC6, recessive, OMIM #216400)

   Marfan syndrome with neonatal progeroid –like lipodystrophy (FBN1, dominant, OMIM #616914)

CAV1-associated neonatal onset lipodystrophy syndrome (dominant)

PCYT1A lipodystrophy (recessive)

   Wiedemann Rautenstrauch syndrome (POLR3A, recessive, OMIM #264090)

   Fontaine progeroid syndrome (SLC25A24, de novo, OMIM # 612289)

  1.3.2 Autoinflammatory syndromes

   PRAAS1 (PSMB8, recessive or digenic with PSMA3 or PSMB4, OMIM #256040)

   PRAAS2 (POMP, dominant, OMIM #618048)

   PRAAS3 (PSMB4, recessive or digenic with PSMB9, OMIM # 617591)

   Panniculitis-associated lipodystrophy (OTULIN, recessive, OMIM #617099)

  1.3.3 Others

   Optic atrophy, cataracts, lipodystrophy/lipoatrophy, peripheral neuropathy (OPA3, dominant, OMIM #165300)

2 Acquired

 2.1 Generalized

  Acquired Generalized Lipodystrophy, idiopathic

  Acquired Generalized Lipodystrophy, autoimmune

  Acquired Generalized Lipodystrophy, panniculitis

 2.2 Partial (excluding HIV associated lipodystrophy)

  Acquired partial lipodystrophy (Barraquer-Simons syndrome)

  Lipodystrophy associated with total body irradiation and hematopoietic stem cell transplant

 2.3 Localized