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Congenital exfoliative erythroderma

MedGen UID:
870446
Concept ID:
C4024892
Disease or Syndrome
HPO: HP:0007381

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital exfoliative erythroderma

Conditions with this feature

Congenital lethal erythroderma
MedGen UID:
384008
Concept ID:
C1856898
Congenital Abnormality
A rare skin disorder characterized by erythrodermic peeling skin from birth with no obvious nail or hair-shaft abnormalities and other associated anomalies including diarrhea, failure to thrive and severe hypoalbuminemia resistant to correction by enteral or intravenous supplementation. An autosomal recessive mode of inheritance is highly probable. The prognosis is poor and infants die in the first months of life. There have been no further descriptions in the literature since 1992.

Professional guidelines

PubMed

Cuperus E, Bygum A, Boeckmann L, Bodemer C, Bolling MC, Caproni M, Diociaiuti A, Emmert S, Fischer J, Gostynski A, Guez S, van Gijn ME, Hannulla-Jouppi K, Has C, Hernández-Martín A, Martinez AE, Mazereeuw-Hautier J, Medvecz M, Neri I, Sigurdsson V, Suessmuth K, Traupe H, Oji V, Pasmans SGMA
J Eur Acad Dermatol Venereol 2022 Jul;36(7):973-986. Epub 2022 Mar 15 doi: 10.1111/jdv.18043. PMID: 35238435Free PMC Article
Cono J, Casey CG, Bell DM; Centers for Disease Control and Prevention
MMWR Recomm Rep 2003 Feb 21;52(RR-4):1-28. PMID: 12617510
Scheimberg I, Hoeger PH, Harper JI, Lake B, Malone M
Pediatr Dev Pathol 2001 May-Jun;4(3):237-45. doi: 10.1007/s100240010171. PMID: 11370261

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