MCID: BNG069
MIFTS: 29

Benign Cephalic Histiocytosis

Categories: Bone diseases, Rare diseases, Skin diseases

Aliases & Classifications for Benign Cephalic Histiocytosis

MalaCards integrated aliases for Benign Cephalic Histiocytosis:

Name: Benign Cephalic Histiocytosis 60 74

Characteristics:

Orphanet epidemiological data:

60
benign cephalic histiocytosis
Inheritance: Not applicable;

Classifications:



External Ids:

ICD10 via Orphanet 35 D76.3
UMLS via Orphanet 75 C0347403
Orphanet 60 ORPHA157997
SNOMED-CT via HPO 70 25694009 443871003
UMLS 74 C0347403

Summaries for Benign Cephalic Histiocytosis

MalaCards based summary : Benign Cephalic Histiocytosis is related to histiocytosis and juvenile xanthogranuloma. An important gene associated with Benign Cephalic Histiocytosis is ITGAL (Integrin Subunit Alpha L), and among its related pathways/superpathways are Phospholipase-C Pathway and Actin Nucleation by ARP-WASP Complex. Affiliated tissues include skin, bone and t cells, and related phenotype is papule.

Wikipedia : 77 Benign cephalic histiocytosis (also known as "Histiocytosis with intracytoplasmic worm-like bodies", and... more...

Related Diseases for Benign Cephalic Histiocytosis

Diseases related to Benign Cephalic Histiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 12)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 10.8
2 juvenile xanthogranuloma 10.2
3 langerhans cell histiocytosis 10.0
4 diabetes mellitus 10.0
5 lymphoma 10.0
6 non-langerhans-cell histiocytosis 10.0
7 diabetes insipidus 10.0
8 indeterminate cell histiocytosis 10.0
9 lymphocytic vasculitis 9.7 ITGAL ITGAX
10 diffuse infiltrative lymphocytosis syndrome 9.7 ITGAL ITGAX
11 lymphoma, non-hodgkin, familial 9.6 ITGAL ITGAX
12 acute promyelocytic leukemia 9.5 ITGAL ITGAX

Graphical network of the top 20 diseases related to Benign Cephalic Histiocytosis:



Diseases related to Benign Cephalic Histiocytosis

Symptoms & Phenotypes for Benign Cephalic Histiocytosis

Human phenotypes related to Benign Cephalic Histiocytosis:

60 33
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 papule 60 33 hallmark (90%) Very frequent (99-80%) HP:0200034

Drugs & Therapeutics for Benign Cephalic Histiocytosis

Search Clinical Trials , NIH Clinical Center for Benign Cephalic Histiocytosis

Genetic Tests for Benign Cephalic Histiocytosis

Anatomical Context for Benign Cephalic Histiocytosis

MalaCards organs/tissues related to Benign Cephalic Histiocytosis:

42
Skin, Bone, T Cells, Monocytes

Publications for Benign Cephalic Histiocytosis

Articles related to Benign Cephalic Histiocytosis:

(show all 39)
# Title Authors Year
1
Treatment of benign cephalic histiocytosis with topical 1% rapamycin ointment. ( 30907021 )
2019
2
Benign cephalic histiocytosis: A case with infiltration of CD1a-positive langerin-negative cells. ( 29430698 )
2018
3
Case of S100-positive benign cephalic histiocytosis involving monocyte/macrophage lineage marker expression. ( 29766556 )
2018
4
Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series. ( 28466548 )
2017
5
Benign cephalic histiocytosis. ( 26125216 )
2015
6
Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis. ( 25526330 )
2014
7
Benign cephalic histiocytosis. ( 25432239 )
2014
8
Large plaque-type benign cephalic histiocytosis showing rapid aggravation following vaccination. ( 25184651 )
2014
9
Benign cephalic histiocytosis. ( 23974593 )
2013
10
Benign cephalic histiocytosis. ( 24350010 )
2013
11
Benign Cephalic Histiocytosis: Case Report and Review of the Literature. ( 23551579 )
2013
12
Case for diagnosis. Benign cephalic histiocytosis. ( 22281921 )
2011
13
Benign cephalic histiocytosis with S-100 protein positivity. ( 21592181 )
2011
14
A case of benign cephalic histiocytosis. ( 22028563 )
2011
15
Benign cephalic histiocytosis: a case report. ( 22148022 )
2011
16
Possible acceleration of regression by antibiotic treatment in benign cephalic histiocytosis with infiltration of CD11a/CD11c+ macrophages. ( 20574620 )
2010
17
Japanese case of benign cephalic histiocytosis. ( 19207442 )
2009
18
Benign cephalic histiocytosis preceding the development of insulin-dependent diabetes mellitus. ( 16445430 )
2006
19
Is disseminated juvenile xanthogranulomatosis benign cephalic histiocytosis? ( 15660896 )
2005
20
Benign cephalic histiocytosis in a British-African child. ( 16190998 )
2005
21
Case 2: Benign cephalic histiocytosis. ( 12139697 )
2002
22
Benign cephalic histiocytosis: a case report and review. ( 12451377 )
2002
23
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? ( 10698221 )
2000
24
Benign cephalic histiocytosis with diabetes insipidus. ( 10990580 )
2000
25
Benign cephalic histiocytosis in Singapore--a review of 8 cases. ( 10709408 )
1999
26
Solitary giant xanthogranuloma and benign cephalic histiocytosis--variants of juvenile xanthogranuloma. ( 7577591 )
1995
27
Facial papules in a child. Benign cephalic histiocytosis. ( 7741553 )
1995
28
Benign cephalic histiocytosis: case report and literature review. ( 8041659 )
1994
29
Benign cephalic histiocytosis--study of a case. ( 8348728 )
1993
30
Multiple eruptive cephalic histiocytomas in a case of T-cell lymphoma. A xanthomatous stage of benign cephalic histiocytosis in an adult patient? ( 8311192 )
1993
31
Benign cephalic histiocytosis: a distinct entity or a part of a wide spectrum of histiocytic proliferative disorders of children? A histopathological study. ( 8214388 )
1993
32
Benign cephalic histiocytosis: a case report. ( 2400727 )
1990
33
Benign cephalic histiocytosis: report of four cases. ( 2508072 )
1989
34
Benign cephalic histiocytosis. ( 2455424 )
1988
35
Benign cephalic histiocytosis. ( 3452310 )
1987
36
Benign cephalic histiocytosis. ( 3740882 )
1986
37
Benign cephalic histiocytosis. ( 3919653 )
1985
38
Benign cephalic histiocytosis. A case report and ultrastructural study. ( 3973127 )
1985
39
Benign cephalic histiocytosis. ( 6721528 )
1984

Variations for Benign Cephalic Histiocytosis

Expression for Benign Cephalic Histiocytosis

Search GEO for disease gene expression data for Benign Cephalic Histiocytosis.

Pathways for Benign Cephalic Histiocytosis

Pathways related to Benign Cephalic Histiocytosis according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.51 ITGAL ITGAX
2
Show member pathways
12.45 ITGAL ITGAX
3
Show member pathways
12.4 ITGAL ITGAX
4
Show member pathways
12.37 ITGAL ITGAX
5
Show member pathways
12.33 ITGAL ITGAX
6
Show member pathways
12.27 ITGAL ITGAX
7
Show member pathways
12.27 ITGAL ITGAX
8
Show member pathways
12.21 ITGAL ITGAX
9 12.1 ITGAL ITGAX
10
Show member pathways
11.88 ITGAL ITGAX
11
Show member pathways
11.86 ITGAL ITGAX
12
Show member pathways
11.7 ITGAL ITGAX
13
Show member pathways
11.68 ITGAL ITGAX
14
Show member pathways
11.66 ITGAL ITGAX
15 11.59 ITGAL ITGAX
16
Show member pathways
11.54 ITGAL ITGAX
17 11.26 ITGAL ITGAX
18 11.22 ITGAL ITGAX
19 11.21 ITGAL ITGAX
20 11 ITGAL ITGAX
21 11 ITGAL ITGAX
22 10.89 ITGAL ITGAX
23 10.89 ITGAL ITGAX
24 10.49 ITGAL ITGAX
25 10.38 ITGAL ITGAX

GO Terms for Benign Cephalic Histiocytosis

Cellular components related to Benign Cephalic Histiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.96 ITGAL ITGAX
2 integrin complex GO:0008305 8.62 ITGAL ITGAX

Biological processes related to Benign Cephalic Histiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.32 ITGAL ITGAX
2 neutrophil degranulation GO:0043312 9.26 ITGAL ITGAX
3 extracellular matrix organization GO:0030198 9.16 ITGAL ITGAX
4 leukocyte migration GO:0050900 8.96 ITGAL ITGAX
5 integrin-mediated signaling pathway GO:0007229 8.62 ITGAL ITGAX

Sources for Benign Cephalic Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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