MCID: BNG069
MIFTS: 28

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 59 72

Characteristics:

Orphanet epidemiological data:

59
benign cephalic histiocytosis
Inheritance: Not applicable;

Classifications:



External Ids:

ICD10 via Orphanet 34 D76.3
UMLS via Orphanet 73 C0347403
Orphanet 59 ORPHA157997
UMLS 72 C0347403

Summaries for Benign Cephalic Histiocytosis

MalaCards based summary : Benign Cephalic Histiocytosis is related to histiocytosis and langerhans cell histiocytosis. 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 phenotypes are papule and skin rash

Wikipedia : 75 Benign cephalic histiocytosis not to be confused with "Neonatal cephalic pustulosis" is a rare skin... 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 16)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 10.9
2 langerhans cell histiocytosis 10.5
3 non-langerhans-cell histiocytosis 10.4
4 juvenile xanthogranuloma 10.4
5 generalized eruptive histiocytosis 10.2
6 histiocytoma 10.0
7 chickenpox 10.0
8 diabetes mellitus 10.0
9 diabetes insipidus 10.0
10 xanthoma disseminatum 10.0
11 papular xanthoma 10.0
12 indeterminate cell histiocytosis 10.0
13 lymphocytic vasculitis 9.5 ITGAX ITGAL
14 diffuse infiltrative lymphocytosis syndrome 9.5 ITGAX ITGAL
15 lymphoma, non-hodgkin, familial 9.4 ITGAX ITGAL
16 acute promyelocytic leukemia 9.2 ITGAX ITGAL

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:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 papule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200034
2 skin rash 59 Very frequent (99-80%)
3 histiocytosis 59 Very frequent (99-80%)
4 inflammatory abnormality of the skin 59 Frequent (79-30%)

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:

41
Skin, Bone, T Cells, Monocytes

Publications for Benign Cephalic Histiocytosis

Articles related to Benign Cephalic Histiocytosis:

(show all 47)
# Title Authors PMID Year
1
Benign cephalic histiocytosis: an atypical case with plaque-like presentation. 38
31302975 2019
2
Treatment of benign cephalic histiocytosis with topical 1% rapamycin ointment. 38
30907021 2019
3
Case of S100-positive benign cephalic histiocytosis involving monocyte/macrophage lineage marker expression. 38
29766556 2018
4
Benign cephalic histiocytosis: A case with infiltration of CD1a-positive langerin-negative cells. 38
29430698 2018
5
Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series. 38
28466548 2017
6
Benign cephalic histiocytosis. 38
26125216 2015
7
Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis. 38
25526330 2014
8
Benign cephalic histiocytosis. 38
25432239 2014
9
Benign cephalic histiocytosis: case report and review of the literature. 38
23551579 2014
10
Large plaque-type benign cephalic histiocytosis showing rapid aggravation following vaccination. 38
25184651 2014
11
Benign cephalic histiocytosis. 38
24350010 2013
12
Benign cephalic histiocytosis. 38
23974593 2013
13
Case for diagnosis. Benign cephalic histiocytosis. 38
22281921 2011
14
Benign cephalic histiocytosis: a case report. 38
22148022 2011
15
Benign cephalic histiocytosis with S-100 protein positivity. 38
21592181 2011
16
A case of benign cephalic histiocytosis. 38
22028563 2011
17
Possible acceleration of regression by antibiotic treatment in benign cephalic histiocytosis with infiltration of CD11a/CD11c+ macrophages. 38
20574620 2010
18
Japanese case of benign cephalic histiocytosis. 38
19207442 2009
19
Benign cephalic histiocytosis preceding the development of insulin-dependent diabetes mellitus. 38
16445430 2006
20
Benign cephalic histiocytosis in a British-African child. 38
16190998 2005
21
Is disseminated juvenile xanthogranulomatosis benign cephalic histiocytosis? 38
15660896 2005
22
Benign cephalic histiocytosis: a case report and review. 38
12451377 2002
23
Case 2: Benign cephalic histiocytosis. 38
12139697 2002
24
Progressive nodular histiocytosis accompanied by systemic disorders. 38
10971342 2000
25
Benign cephalic histiocytosis with diabetes insipidus. 38
10990580 2000
26
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? 38
10698221 2000
27
Benign cephalic histiocytosis in Singapore--a review of 8 cases. 38
10709408 1999
28
[Acquired regressive cutaneous non-Langerhans-cell histiocytosis in an infant]. 38
9740830 1997
29
Non-Langerhans cell histiocytoses. A new unifying concept. 38
8902096 1996
30
Solitary giant xanthogranuloma and benign cephalic histiocytosis--variants of juvenile xanthogranuloma. 38
7577591 1995
31
Facial papules in a child. Benign cephalic histiocytosis. 38
7741553 1995
32
Benign cephalic histiocytosis: case report and literature review. 38
8041659 1994
33
Multiple eruptive cephalic histiocytomas in a case of T-cell lymphoma. A xanthomatous stage of benign cephalic histiocytosis in an adult patient? 38
8311192 1993
34
Benign cephalic histiocytosis: a distinct entity or a part of a wide spectrum of histiocytic proliferative disorders of children? A histopathological study. 38
8214388 1993
35
Benign cephalic histiocytosis--study of a case. 38
8348728 1993
36
[A case of benign cephalic histiocytosis]. 38
1886280 1991
37
Benign cephalic histiocytosis: a case report. 38
2400727 1990
38
Benign cephalic histiocytosis: report of four cases. 38
2508072 1989
39
Eruptive histiocytoma. 38
2541184 1989
40
Benign cephalic histiocytosis. 38
2455424 1988
41
Benign cephalic histiocytosis. 38
3452310 1987
42
Benign cephalic histiocytosis. 38
3740882 1986
43
Cutaneous histiocytosis syndromes. 38
2999740 1985
44
Benign cephalic histiocytosis. 38
3919653 1985
45
Benign cephalic histiocytosis. A case report and ultrastructural study. 38
3973127 1985
46
Benign cephalic histiocytosis. 38
6721528 1984
47
[Self-healing childhood histiocytosis X (Illig-Fanconi disease). Comments on ultrastructural aspects and etiopathogenic classification of histiocytosis]. 38
6764232 1982

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 ITGAX ITGAL
2
Show member pathways
12.45 ITGAX ITGAL
3
Show member pathways
12.4 ITGAX ITGAL
4
Show member pathways
12.37 ITGAX ITGAL
5
Show member pathways
12.33 ITGAX ITGAL
6
Show member pathways
12.27 ITGAX ITGAL
7
Show member pathways
12.27 ITGAX ITGAL
8
Show member pathways
12.21 ITGAX ITGAL
9 12.1 ITGAX ITGAL
10
Show member pathways
11.86 ITGAX ITGAL
11
Show member pathways
11.7 ITGAX ITGAL
12
Show member pathways
11.69 ITGAX ITGAL
13
Show member pathways
11.66 ITGAX ITGAL
14
Show member pathways
11.65 ITGAX ITGAL
15 11.59 ITGAX ITGAL
16
Show member pathways
11.56 ITGAX ITGAL
17 11.33 ITGAX ITGAL
18 11.22 ITGAX ITGAL
19 11.21 ITGAX ITGAL
20 11 ITGAX ITGAL
21 10.94 ITGAX ITGAL
22 10.89 ITGAX ITGAL
23 10.87 ITGAX ITGAL
24 10.49 ITGAX ITGAL
25 10.38 ITGAX ITGAL

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 ITGAX ITGAL
2 integrin complex GO:0008305 8.62 ITGAX ITGAL

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 ITGAX ITGAL
2 neutrophil degranulation GO:0043312 9.26 ITGAX ITGAL
3 extracellular matrix organization GO:0030198 9.16 ITGAX ITGAL
4 leukocyte migration GO:0050900 8.96 ITGAX ITGAL
5 integrin-mediated signaling pathway GO:0007229 8.62 ITGAX ITGAL

Sources for Benign Cephalic Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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