Table 1. List of lipodystrophy subformes (adapted from [3]).

1 Congenital (Familial)

 1.1 Generalized (CGL)

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

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

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

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

  1.1.5 PPARG -associated CGL (PPARG, recessive)

  1.1.6 CGL with progressive encephalopathy (OMIM: #615924)

  1.1.7 CGL undefined

 1.2 Partial

  1.2.1 Type 1 FPLD (Köbberling syndrome; genes unknown, OMIM %608600)

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

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

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

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

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

  1.2.7 Type 7 FPLD with congenital cataracts, and neurodegeneration (CAV1, dominant, OMIM

  1.2.8 AKT2-linked lipodystrophy (AKT2, dominant)

  1.2.9 MFN2 associated FPLD (MFN2)

 1.3 Systemic

  1.3.1 Progeroid syndromes

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

   1.3.1.2 Atypical Werner syndrome and atypical progeroid syndrome (LMNA-associated)

   1.3.1.3 SHORT syndrome (PIK3R1, dominant, OMIM #269880)

   1.3.1.4 MDPL syndrome (generalized or partial; POLD1, dominant, OMIM #615381)

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

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

   1.3.1.7 Mandibuloacral dysplasia, type B (ZMPSTE24, recessive, OMIM #608612)

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

   1.3.1.9 Cockayne syndrome type B (ERCC6, recessive, OMIM #133540)

   1.3.1.10 Cockayne syndrome type A (ERCC8, recessive, OMIM #216400)

   1.3.1.11 Lipodystrophy-intellectual disability-deafness syndrome (Rajab-Spranger syndrome, OMIM %608154)

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

   1.3.1.13 CAV1-associated neonatal onset lipodystrophy syndrome (CAV1, dominant)

   1.3.1.14 Werner syndrome (WRN/RECQL2, recessive, OMIM #277700)

   1.3.1.15 Mandibuloacral dysplasia type A (LMNA, recessive, OMIM #248370)

   1.3.1.16 PCYT1A lipodystrophy (PCYT1A, recessive)

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

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

  1.3.2 Autoinflammatory syndromes, ALDD (generalized or partial)

   1.3.2.1 PRAAS 1 (PSMB8, recessive or digenic with PSMA3 or PSMB4, OMIM #256040)

   1.3.2.2 PRAAS 2 (POMP, dominant, OMIM #618048)

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

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

  1.3.3 Others

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

2 Acquired

 2.1 Generalized

  2.1.1 Acquired generalized lipodystrophy, idiopathic

  2.1.2 Acquired generalized lipodystrophy, autoimmune

  2.1.3 Acquired generalized lipodystrophy, panniculitis

 2.2 Partial (excluding HIV associated lipodystrophy)

  2.2.1 Acquired partial lipodystrophy (Barraquer-Simons syndrome, OMIM #608709)

  2.2.2 Lipodystrophy associated with total body irradiation and hematopoietic stem cell transplant

  2.2.3 Acquired partial lipodystrophy, undefined

 2.3 Localized

  2.3.1 Lipodystrophy caused by drug injections

  2.3.2 Lipodystrophy semicircularis

  2.3.3 Centrifugal lipodystrophy

  2.3.4 Progressive hemifacial atrophy (Parry Romberg syndrome, OMIM % 141300)

  2.3.5 Idiopathic localized lipodystrophy

  2.3.6 Panniculitis induced localized lipodystrophy