MCID: DRM050
MIFTS: 27

Dermographism, Familial

Categories: Genetic diseases, Rare diseases, Skin diseases

Aliases & Classifications for Dermographism, Familial

MalaCards integrated aliases for Dermographism, Familial:

Name: Dermographism, Familial 58
Familial Dermographism 54 74
Dermo-Distortive Urticaria 54
Dermodistortive Urticaria 74
Dermatographism, Familial 58
Symptomatic Dermographism 74
Familial Dermatographism 54
Dermatographia 54
Dermographism 54

Characteristics:

OMIM:

58
Inheritance:
autosomal dominant


HPO:

33
dermographism, familial:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM 58 125635
MedGen 43 C1852145

Summaries for Dermographism, Familial

NIH Rare Diseases : 54 Familial dermographism is a condition also known as skin writing. When people who have dermatographia lightly scratch their skin, the scratches redden into a raised wheal similar to hives. Signs and symptoms of dermatographia include raised red lines, swelling, inflammation, hive-like welts and itching. Symptoms usually disappear within 30 minutes. The exact cause of this condition is unknown. Treatment may invovle use of antihistamines if symptoms do not go away on their own.

MalaCards based summary : Dermographism, Familial, also known as familial dermographism, is related to dermatographia and physical urticaria, and has symptoms including integumentary system signs and symptoms and darier's sign. An important gene associated with Dermographism, Familial is PRG2 (Proteoglycan 2, Pro Eosinophil Major Basic Protein). The drugs Omalizumab and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include skin, and related phenotypes are stroke and dermatographic urticaria

Wikipedia : 77 Dermatographic urticaria is a skin disorder and one of the most common types of urticaria, affecting... more...

Description from OMIM: 125635

Related Diseases for Dermographism, Familial

Diseases related to Dermographism, Familial via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 26)
# Related Disease Score Top Affiliating Genes
1 dermatographia 12.4
2 physical urticaria 11.5
3 urticaria 10.6
4 vibratory urticaria 10.3
5 mastocytosis, cutaneous 10.2
6 allergic conjunctivitis 10.1
7 conjunctivitis 10.1
8 mastocytosis 10.1
9 cold urticaria 10.1
10 ige responsiveness, atopic 10.0
11 alopecia universalis congenita 10.0
12 alopecia 10.0
13 systemic scleroderma 10.0
14 latex allergy 10.0
15 dermatomyositis 10.0
16 adult-onset still's disease 10.0
17 dermatitis 10.0
18 contact dermatitis 10.0
19 allergic contact dermatitis 10.0
20 cellulitis 10.0
21 wells syndrome 10.0
22 chronic actinic dermatitis 10.0
23 fixed drug eruption 10.0
24 atopic keratoconjunctivitis 9.7 IGHE PRG2
25 hypereosinophilic syndrome 9.6 IGHE PRG2
26 asthma 9.5 IGHE PRG2

Graphical network of the top 20 diseases related to Dermographism, Familial:



Diseases related to Dermographism, Familial

Symptoms & Phenotypes for Dermographism, Familial

Human phenotypes related to Dermographism, Familial:

33
# Description HPO Frequency HPO Source Accession
1 stroke 33 HP:0001297
2 dermatographic urticaria 33 HP:0011971

Symptoms via clinical synopsis from OMIM:

58
Skin:
dermographism
localized urticarial wheal and flare from a single skin stroke or scratch

Clinical features from OMIM:

125635

UMLS symptoms related to Dermographism, Familial:


integumentary system signs and symptoms, darier's sign

Drugs & Therapeutics for Dermographism, Familial

Drugs for Dermographism, Familial (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Omalizumab Approved, Investigational Phase 2 242138-07-4
2 Immunoglobulins Phase 2
3 Anti-Allergic Agents Phase 2
4 Respiratory System Agents Phase 2
5 Anti-Asthmatic Agents Phase 2
6 Antibodies, Monoclonal Phase 2
7 Immunologic Factors Phase 2
8 Antibodies Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Urticaria Facticia Treatment With Omalizumab (UFO) Completed NCT02169115 Phase 2 Omalizumab;Omalizumab;Placebo

Search NIH Clinical Center for Dermographism, Familial

Genetic Tests for Dermographism, Familial

Anatomical Context for Dermographism, Familial

MalaCards organs/tissues related to Dermographism, Familial:

42
Skin

Publications for Dermographism, Familial

Articles related to Dermographism, Familial:

# Title Authors Year
1
Familial dermographism. ( 2063925 )
1991

Variations for Dermographism, Familial

Expression for Dermographism, Familial

Search GEO for disease gene expression data for Dermographism, Familial.

Pathways for Dermographism, Familial

GO Terms for Dermographism, Familial

Biological processes related to Dermographism, Familial according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 8.96 IGHE PRG2
2 defense response to bacterium GO:0042742 8.62 IGHE PRG2

Sources for Dermographism, Familial

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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