Class information for:
Level 1: SJOGREN LARSSON SYNDROME//HARLEQUIN ICHTHYOSIS//LAMELLAR ICHTHYOSIS

Basic class information
ID Publications Average number
of references		 Avg. shr. active
ref. in WoS
14082 729 27.0 68%


Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)


ID, lev.
above		 Publications Label for level above
800 11412 EPIDERMOLYSIS BULLOSA//TRANSGLUTAMINASES//INTERMEDIATE FILAMENTS

Terms with highest relevance score


Rank Term Type of term Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class
1 SJOGREN LARSSON SYNDROME Author keyword 105 89% 7% 48
2 HARLEQUIN ICHTHYOSIS Author keyword 102 95% 5% 35
3 LAMELLAR ICHTHYOSIS Author keyword 79 82% 6% 46
4 ICHTHYOSIS Author keyword 33 30% 13% 94
5 AUTOSOMAL RECESSIVE CONGENITAL ICHTHYOSIS Author keyword 23 86% 2% 12
6 FATTY ALDEHYDE DEHYDROGENASE Author keyword 21 78% 2% 14
7 COLLODION BABY Author keyword 16 61% 2% 17
8 CONGENITAL ICHTHYOSIFORM ERYTHRODERMA Author keyword 13 80% 1% 8
9 ABCA12 Author keyword 13 71% 1% 10
10 CONGENITAL ICHTHYOSIS Author keyword 12 53% 2% 16

Web of Science journal categories

Author Key Words


Rank Web of Science journal category Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class		 LCSH search Wikipedia search
1 SJOGREN LARSSON SYNDROME 105 89% 7% 48 Search SJOGREN+LARSSON+SYNDROME Search SJOGREN+LARSSON+SYNDROME
2 HARLEQUIN ICHTHYOSIS 102 95% 5% 35 Search HARLEQUIN+ICHTHYOSIS Search HARLEQUIN+ICHTHYOSIS
3 LAMELLAR ICHTHYOSIS 79 82% 6% 46 Search LAMELLAR+ICHTHYOSIS Search LAMELLAR+ICHTHYOSIS
4 ICHTHYOSIS 33 30% 13% 94 Search ICHTHYOSIS Search ICHTHYOSIS
5 AUTOSOMAL RECESSIVE CONGENITAL ICHTHYOSIS 23 86% 2% 12 Search AUTOSOMAL+RECESSIVE+CONGENITAL+ICHTHYOSIS Search AUTOSOMAL+RECESSIVE+CONGENITAL+ICHTHYOSIS
6 FATTY ALDEHYDE DEHYDROGENASE 21 78% 2% 14 Search FATTY+ALDEHYDE+DEHYDROGENASE Search FATTY+ALDEHYDE+DEHYDROGENASE
7 COLLODION BABY 16 61% 2% 17 Search COLLODION+BABY Search COLLODION+BABY
8 CONGENITAL ICHTHYOSIFORM ERYTHRODERMA 13 80% 1% 8 Search CONGENITAL+ICHTHYOSIFORM+ERYTHRODERMA Search CONGENITAL+ICHTHYOSIFORM+ERYTHRODERMA
9 ABCA12 13 71% 1% 10 Search ABCA12 Search ABCA12
10 CONGENITAL ICHTHYOSIS 12 53% 2% 16 Search CONGENITAL+ICHTHYOSIS Search CONGENITAL+ICHTHYOSIS

Key Words Plus


Rank Web of Science journal category Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class
1 RECESSIVE CONGENITAL ICHTHYOSIS 61 71% 7% 49
2 LAMELLAR ICHTHYOSIS 56 40% 15% 110
3 HARLEQUIN ICHTHYOSIS 43 50% 9% 62
4 ERYTHRODERMA 28 38% 8% 60
5 KERATINOCYTE TRANSGLUTAMINASE 25 36% 8% 55
6 ABCA12 23 70% 3% 19
7 ABNORMAL LAMELLAR GRANULES 23 100% 1% 10
8 TRANSPORTER ABCA12 23 62% 3% 24
9 FALDH GENE 18 89% 1% 8
10 CONGENITAL ICHTHYOSIS 17 50% 3% 24

Journals

Reviews


Title Publ. year Cit. Active references % act. ref.
to same field
Harlequin Ichthyosis A Review of Clinical and Molecular Findings in 45 Cases 2011 28 22 86%
Sjogren-Larsson syndrome: Molecular genetics and biochemical pathogenesis of fatty aldehyde dehydrogenase deficiency 2007 47 64 81%
The roles of ABCA12 in epidermal lipid barrier formation and keratinocyte differentiation 2014 6 46 57%
ABCA12 Mutations and Autosomal Recessive Congenital Ichthyosis: A Review of Genotype/Phenotype Correlations and of Pathogenetic Concepts 2010 27 44 84%
Transglutaminase-1 Gene Mutations in Autosomal Recessive Congenital Ichthyosis: Summary of Mutations (Including 23 Novel) and Modeling of TGase-1 2009 28 95 59%
Harlequin ichthyosis and other autosomal recessive congenital ichthyoses: The underlying genetic defects and pathomechanisms 2006 36 44 80%
Sjogren-Larsson syndrome 2007 22 23 96%
Harlequin ichthyosis: ABCA12 mutations underlie defective lipid transport, reduced protease regulation and skin-barrier dysfunction 2013 10 52 48%
The clinical spectrum of nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis 2003 42 27 100%
Fatty aldehyde and fatty alcohol metabolism: Review and importance for epidermal structure and function 2014 6 135 30%

Address terms


Rank Address term Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ.
in class
1 DERMATOGENET 4 36% 1.2% 9
2 DERMATOL DNA 3 60% 0.4% 3
3 ULTRASTRUCT SKIN 1 38% 0.4% 3
4 ABT DERMATOGENET 1 100% 0.3% 2
5 REFERENCE MALAD GENET EXP S CUTANEE MA 1 40% 0.3% 2
6 REFERENCE RARE SKIN DIS 1 21% 0.5% 4
7 BARTS LONDON MED DENTISTRY 1 50% 0.1% 1
8 CHU NECKER SICK CHILDREN 1 50% 0.1% 1
9 CNRS UMR 7225UMR S 1127 CERVEAU MOELLE EP 1 50% 0.1% 1
10 CNRSUMR6061 1 50% 0.1% 1

Related classes at same level (level 1)


Rank Relatedness score Related classes
1 0.0000183867 X LINKED ICHTHYOSIS//STEROID SULFATASE DEFICIENCY//STS GENE
2 0.0000123829 PACHYONYCHIA CONGENITA//EPIDERMOLYSIS BULLOSA SIMPLEX//EPIDERMOLYTIC HYPERKERATOSIS
3 0.0000101253 INVOLUCRIN//SMALL PROLINE RICH PROTEINS//SMALL PROLINE RICH PROTEIN
4 0.0000097386 NETHERTON SYNDROME//KALLIKREINS//KALLIKREIN RELATED PEPTIDASES
5 0.0000079717 STRATUM CORNEUM//TRANSEPIDERMAL WATER LOSS//CERAMIDES
6 0.0000073311 GAPO SYNDROME//IFAP SYNDROME//ICHTHYOSIS FOLLICULARIS
7 0.0000070187 KERATOSIS LICHENOIDES CHRONICA//AINHUM//OLMSTED SYNDROME
8 0.0000066323 EPIDERMOLYSIS BULLOSA//DYSTROPHIC EPIDERMOLYSIS BULLOSA//COL7A1
9 0.0000062649 TRANSGLUTAMINASES//TRANSGLUTAMINASE//TISSUE TRANSGLUTAMINASE
10 0.0000060568 ALDEHYDE DEHYDROGENASE//ALDH3A1//MOL TOXICOL ENVIRONM HLTH SCI PROGRAM