BZX
MCID: BZX001
MIFTS: 43

Bazex Syndrome (BZX)

Categories: Cancer diseases, Fetal diseases, Rare diseases, Skin diseases

Aliases & Classifications for Bazex Syndrome

MalaCards integrated aliases for Bazex Syndrome:

Name: Bazex Syndrome 57 53 59 13
Follicular Atrophoderma and Basal Cell Carcinomas 57 53 59
Bazex-Dupre-Christol Syndrome 57 53 59
Bdcs 57 53 59
Bazex-Dupré-Christol Syndrome 75 53
Bzx 57 53
Follicular Atrophoderma-Basocellular Proliferations-Hypotrichosis Syndrome 53
Follicular Atrophoderma-Basal Cell Carcinoma Syndrome 53
Follicular Atrophoderma and Basal Cell Epitheliomata 72
Follicular Atrophoderma-Basal Cell Carcinoma 59
Acrokeratosis Paraneoplastica of Bazex 59
Bazex-Dupre-Christol Syndrome; Bdcs 57
Acrokeratosis Paraneoplastica 59
Acrokeratosis of Bazex 59

Characteristics:

Orphanet epidemiological data:

59
bazex syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult;
bazex-dupre-christol syndrome
Inheritance: X-linked dominant; Prevalence: <1/1000000 (Worldwide); Age of onset: Infancy,Neonatal;

OMIM:

57
Miscellaneous:
onset in infancy or early childhood
basal cell neoplasms develop after second decade

Inheritance:
x-linked dominant


HPO:

32
bazex syndrome:
Onset and clinical course infantile onset
Inheritance x-linked dominant inheritance


Classifications:



External Ids:

OMIM 57 301845
MESH via Orphanet 45 C537663
ICD10 via Orphanet 34 L98.8
UMLS via Orphanet 73 C0346104 C0406355
MedGen 42 C0346104
UMLS 72 C0346104

Summaries for Bazex Syndrome

NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 113DefinitionBazex-Dupre-Christol syndrome is a rare genodermatosis with a predisposition to early-onset basal cell carcinomas.EpidemiologySo far, 143 cases have been reported, mostly from France and Belgium.Clinical descriptionThe disease manifests during the neonatal period or during infancy. It is characterized by hypotrichosis, hypohidrosis, milia and basal cell carcinomas of early onset. Follicular atrophoderma is frequent and most common on the dorsum of the hands and feet, the extensor surfaces of the elbows and knees, and the face. Hypotrichosis affects the scalp and sometimes the eyebrows. The milia papules and basal cell carcinomas are located predominantly on the face. Basal cell carcinomas develop in 40% of patients, usually during the 2nd or 3rd decade of life. Additional common features include basal cell hamartomas, trichoepitheliomas and, in very rare cases, atopy, keratosis pilaris, ichthyosis, arachnodactyly with joint hyperlaxity, osteochondritis, deafness and learning difficulties.EtiologyThe gene has been mapped to the long arm of the X chromosome, within the Xq24-q27.1 region. UBE2A (Xq24), encoding a protein involved in repair of UV-damaged DNA, has been proposed as a candidate gene.Differential diagnosisThe differential diagnosis should include Gorlin syndrome, which also leads to multiple basal cell carcinomas of early onset, and X-linked dominant chondrodysplasia punctata (see these terms) in which follicular atrophoderma may also be observed. Rombo syndrome and generalized basaloid follicular hamartoma syndrome (see these terms) should also be included in the differential diagnosis.Genetic counselingTransmission is X-linked dominant.Management and treatmentManagement involves photoprotection and early detection of basal cell carcinomas. Surgical intervention, and sometimes cryosurgery or topical imiquimod, are indicated for basal cell carcinomas. Radiotherapy is contraindicated.Visit the Orphanet disease page for more resources.

MalaCards based summary : Bazex Syndrome, also known as follicular atrophoderma and basal cell carcinomas, is related to brachydactyly, type c and collecting duct carcinoma. An important gene associated with Bazex Syndrome is BZX (Bazex Syndrome), and among its related pathways/superpathways are G-protein signaling_RhoA regulation pathway and Lung fibrosis. Affiliated tissues include skin, lung and breast, and related phenotypes are coarse hair and palmoplantar keratoderma

OMIM : 57 Bazex syndrome is an X-linked dominant disorder characterized by a triad of congenital hypotrichosis, follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows or knees, and the development of basal cell neoplasms, including basal cell nevi and basal cell carcinomas from the second decade onward (Yung and Newton-Bishop, 2005). Rombo syndrome (180730) has similar features, but shows autosomal dominant inheritance. (301845)

Wikipedia : 75 Bazex-Dupre-Christol syndrome is a very rare condition inherited in an X-linked dominant fashion.... more...

Related Diseases for Bazex Syndrome

Diseases related to Bazex Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 94, show less)
# Related Disease Score Top Affiliating Genes
1 brachydactyly, type c 11.6
2 collecting duct carcinoma 11.4
3 pili torti, early-onset 11.2
4 basal cell carcinoma 1 11.2
5 pili torti 11.2
6 erythrokeratoderma ''en cocardes'' 11.0
7 rare genetic skin disease 10.7
8 attention deficit-hyperactivity disorder 10.6
9 chondrodysplasia punctata syndrome 10.6
10 miliaria rubra 10.6
11 miliaria 10.6
12 ectodermal dysplasia 10.6
13 hidradenitis suppurativa 10.6
14 hidradenitis 10.6
15 skin carcinoma 10.6
16 follicular basal cell carcinoma 10.6
17 folliculitis 10.6
18 bile duct cysts 10.4
19 squamous cell carcinoma 10.3
20 acanthosis nigricans 10.2
21 bladder cancer 10.2
22 carpal tunnel syndrome 10.2
23 systemic lupus erythematosus 10.2
24 ovarian cancer 10.2
25 nail disorder, nonsyndromic congenital, 10 10.2
26 larynx cancer 10.2
27 thymic carcinoma 10.2
28 liposarcoma 10.2
29 hypertrichosis 10.2
30 lupus erythematosus 10.2
31 dermatophytosis 10.2
32 pemphigus 10.2
33 retroperitoneal liposarcoma 10.2
34 dysphagia 10.2
35 paraneoplastic pemphigus 10.2
36 dermatitis 10.2
37 adenocarcinoma 10.2
38 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.2
39 carcinosarcoma 10.2
40 ovarian epithelial cancer 10.2
41 cholangiocarcinoma 10.2
42 intrahepatic cholangiocarcinoma 10.2
43 lichen planus pigmentosus 10.2
44 keratosis 10.2
45 skin atrophy 10.2
46 skin disease 10.2
47 breast cancer 10.1
48 porphyria cutanea tarda 10.1
49 anhidrosis 10.1
50 porphyria 10.1
51 ichthyosis 10.1
52 lung squamous cell carcinoma 10.1
53 pustulosis of palm and sole 10.1
54 hypotrichosis 10.1
55 psoriasis 10.1
56 lichen planus 10.1
57 paraneoplastic syndromes 10.1
58 colorectal cancer 9.9
59 hepatocellular carcinoma 9.9
60 esophageal cancer 9.9
61 dowling-degos disease 1 9.9
62 temporal arteritis 9.9
63 vitiligo-associated multiple autoimmune disease susceptibility 6 9.9
64 lung cancer 9.9
65 pulmonary alveolar microlithiasis 9.9
66 suppression of tumorigenicity 12 9.9
67 vitiligo-associated multiple autoimmune disease susceptibility 1 9.9
68 lung cancer susceptibility 3 9.9
69 gastric cancer 9.9
70 hydrops, lactic acidosis, and sideroblastic anemia 9.9
71 exanthem 9.9
72 peripheral t-cell lymphoma 9.9
73 tongue squamous cell carcinoma 9.9
74 follicular lymphoma 9.9
75 lymphoma 9.9
76 dermatomyositis 9.9
77 paronychia 9.9
78 colon adenocarcinoma 9.9
79 basal cell carcinoma 9.9
80 synovitis 9.9
81 epidermolysis bullosa 9.9
82 palmoplantar keratosis 9.9
83 gastric adenocarcinoma 9.9
84 cervical squamous cell carcinoma 9.9
85 epidermolysis bullosa acquisita 9.9
86 connective tissue disease 9.9
87 bullous pemphigoid 9.9
88 hodgkin's lymphoma, lymphocytic-histiocytic predominance 9.9
89 alopecia areata 9.9
90 alopecia 9.9
91 hypereosinophilic syndrome 9.9
92 ichthyosis, acquired 9.9
93 remitting seronegative symmetrical synovitis with pitting edema 9.9
94 bullous lichen planus 9.9

Graphical network of the top 20 diseases related to Bazex Syndrome:



Diseases related to Bazex Syndrome

Symptoms & Phenotypes for Bazex Syndrome

Human phenotypes related to Bazex Syndrome:

59 32 (showing 45, show less)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 coarse hair 59 32 hallmark (90%) Very frequent (99-80%) HP:0002208
2 palmoplantar keratoderma 59 32 hallmark (90%) Very frequent (99-80%) HP:0000982
3 parakeratosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001036
4 nail dystrophy 59 32 hallmark (90%) Very frequent (99-80%) HP:0008404
5 yellow nails 59 32 hallmark (90%) Very frequent (99-80%) HP:0011367
6 scaling skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0040189
7 subcutaneous nodule 59 32 frequent (33%) Frequent (79-30%) HP:0001482
8 anemia 59 32 frequent (33%) Frequent (79-30%) HP:0001903
9 abnormal blistering of the skin 59 32 frequent (33%) Frequent (79-30%) HP:0008066
10 liposarcoma 59 32 frequent (33%) Frequent (79-30%) HP:0012034
11 lip hyperpigmentation 59 32 frequent (33%) Frequent (79-30%) HP:0100816
12 sparse hair 59 32 frequent (33%) Frequent (79-30%) HP:0008070
13 basal cell carcinoma 59 32 frequent (33%) Frequent (79-30%) HP:0002671
14 trichorrhexis nodosa 59 32 frequent (33%) Frequent (79-30%) HP:0009886
15 milia 59 32 frequent (33%) Frequent (79-30%) HP:0001056
16 hypotrichosis of the scalp 59 32 frequent (33%) Frequent (79-30%) HP:0004782
17 pili torti 59 32 frequent (33%) Frequent (79-30%) HP:0003777
18 sparse or absent eyelashes 59 32 frequent (33%) Frequent (79-30%) HP:0200102
19 sparse and thin eyebrow 32 frequent (33%) HP:0000535
20 macrotia 59 32 occasional (7.5%) Occasional (29-5%) HP:0000400
21 exostoses 59 32 occasional (7.5%) Occasional (29-5%) HP:0100777
22 acanthosis nigricans 59 32 occasional (7.5%) Occasional (29-5%) HP:0000956
23 edema 59 32 occasional (7.5%) Occasional (29-5%) HP:0000969
24 hypoplasia of the ear cartilage 59 32 occasional (7.5%) Occasional (29-5%) HP:0100720
25 abnormality of finger 59 32 occasional (7.5%) Occasional (29-5%) HP:0001167
26 abnormality of the clavicle 59 32 occasional (7.5%) Occasional (29-5%) HP:0000889
27 eczema 32 occasional (7.5%) HP:0000964
28 acne inversa 32 occasional (7.5%) HP:0040154
29 trichoepithelioma 32 occasional (7.5%) HP:0025367
30 pruritus 59 32 very rare (1%) Very rare (<4-1%) HP:0000989
31 lung adenocarcinoma 59 32 very rare (1%) Very rare (<4-1%) HP:0030078
32 hyperkeratosis 59 Very frequent (99-80%)
33 neoplasm 59 Very frequent (99-80%)
34 hypohidrosis 32 HP:0000966
35 nevus 32 HP:0003764
36 underdeveloped nasal alae 32 HP:0000430
37 abnormality of the mouth 32 HP:0000153
38 joint hypermobility 32 HP:0001382
39 hypotrichosis 32 HP:0001006
40 hyperpigmentation of the skin 32 HP:0000953
41 sparse eyebrow 59 Frequent (79-30%)
42 atopic dermatitis 32 HP:0001047
43 narrow nasal ridge 32 HP:0000418
44 low hanging columella 32 HP:0009765
45 keratosis pilaris 32 HP:0032152

Symptoms via clinical synopsis from OMIM:

57
Skeletal:
joint hypermobility

Skin Nails Hair Skin:
atopic dermatitis
keratosis pilaris
facial milia
facial hyperpigmentation
follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows and knees
more
Head And Neck Nose:
prominent columella
hypoplastic nasal alae
pinched nose
long, thin nose

Head And Neck Mouth:
lingua plicata

Skin Nails Hair Hair:
hypotrichosis
trichorrhexis nodosa
pili torti
dry, coarse hair
hair shaft abnormalities

Neoplasia:
basal cell nevi
basal cell carcinomas
basocellular neoplasms
basocellular neoplasms develop after second decade

Head And Neck Face:
facial milia
facial hyperpigmentation

Clinical features from OMIM:

301845

GenomeRNAi Phenotypes related to Bazex Syndrome according to GeneCards Suite gene sharing:

26 (showing 4, show less)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased Sindbis virus (SIN) capsid and autophagosome LC3 protein colocalization GR00242-A-1 9.56 ACTRT1 FGF7
2 Decreased Sindbis virus (SIN) capsid and autophagosome LC3 protein colocalization GR00242-A-2 9.56 ACTRT1 FGF7
3 Decreased mitophagy mCherry-Parkin protein expression after carbonyl cyanide m-chlorphenylhydrazone (CCCP) stimulation GR00242-A-4 9.16 ACTRT1 FGF7
4 Decreased viability after sindbis virus (SIN) dsTE12Q infection GR00242-A-3 8.62 ACTRT1 FGF7

Drugs & Therapeutics for Bazex Syndrome

Interventional clinical trials:

(showing 1, show less)
# Name Status NCT ID Phase Drugs
1 Evaluation of the Effect of Planned Discharge Training on Health Knowledge and Beliefs on Patients With Acute Myocardial Infarction Completed NCT03985397

Search NIH Clinical Center for Bazex Syndrome

Genetic Tests for Bazex Syndrome

Anatomical Context for Bazex Syndrome

MalaCards organs/tissues related to Bazex Syndrome:

41
Skin, Lung, Breast, Colon, Prostate, T Cells

Publications for Bazex Syndrome

Articles related to Bazex Syndrome:

(showing 118, show less)
# Title Authors PMID Year
1
Overlap between the Bazex syndrome and congenital hypotrichosis and milia. 38 8
7677860 1995
2
Follicular atrophoderma and basal cell carcinomas: the Bazex syndrome. 38 8
879818 1977
3
A case of Bazex-Dupré-Christol syndrome associated with multiple genital trichoepitheliomas. 8
16120174 2005
4
Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts. 8
10025974 1998
5
A Scottish family with Bazex-Dupré-Christol syndrome: follicular atrophoderma, congenital hypotrichosis, and basal cell carcinoma. 8
8782050 1996
6
The gene for Bazex-Dupré-Christol syndrome maps to chromosome Xq. 8
7615983 1995
7
Congenital hypotrichosis and milia: report of a large family suggesting X-linked dominant inheritance. 8
7747764 1994
8
Bazex-Dupré-Christol syndrome: a possible diagnosis for basal cell carcinomas, coarse sparse hair, and milia. 8
8456866 1993
9
Basal cell carcinomas, coarse sparse hair, and milia. 8
1642265 1992
10
Follicular atrophoderma with multiple basal cell carcinomas (Bazex). 8
708616 1978
11
Studies of the density and the properties of the hair in a new inherited syndrome of hypotrichosis. 8
4220381 1972
12
[Follicular atrophoderma, basocellular proliferations and hypotrichosis]. 8
5126848 1971
13
[Follicular atrophodermia, epidermal cysts and hypotrichosis. Apropos of a familial case of genodermatosis]. 8
5585206 1967
14
[Follicular atrophoderma, baso-cellular proliferations and hypotrichosis]. 8
5963641 1966
15
Acrokeratosis paraneoplastica (Bazex syndrome) with bullous lesions. 38
31339109 2019
16
Synchronous Occurrence of Bazex Syndrome and Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome in a Patient with Lung Cancer. 38
31292397 2019
17
Acrokeratosis paraneoplastica (Bazex syndrome) associated with peripheral T-cell lymphoma. 38
30581941 2019
18
Bazex syndrome in a patient with head and neck malignancy. 38
29846313 2018
19
Bazex Syndrome. 38
29279508 2018
20
Acrokeratosis paraneoplastica (Bazex syndrome). 38
29036561 2018
21
Is it just a psoriasiform dermatitis? 38
29447642 2017
22
Acrokeratosis paraneoplastica (Bazex syndrome). 38
29121372 2017
23
Paraneoplastic acrokeratosis (Bazex syndrome): unusual association with in situ follicular lymphoma and response to acitretin. 38
28961299 2017
24
Acrokeratosis paraneoplastica (Bazex syndrome) - a systematic review on risk factors, diagnosis, prognosis and management. 38
28273374 2017
25
Bazex syndrome. 38
28461377 2017
26
Bazex Syndrome in Lung Squamous Cell Carcinoma: High Expression of Epidermal Growth Factor Receptor in Lesional Keratinocytes with Th2 Immune Shift. 38
28101024 2016
27
Acitretin amelioration of Acrokeratosis Paraneoplastica (Bazex Syndrome) in cases of incurable squamous cell carcinoma of the hypopharynx. 38
28329620 2016
28
Bazex Syndrome with Hypoalbuminemia and Severe Ascites. 38
27721759 2016
29
Acrokeratosis Paraneoplastica Associated with Cervical Squamous Cell Carcinoma. 38
28101384 2016
30
Acrokeratosis paraneoplastica (Bazex syndrome) associated with metastatic cutaneous squamous cell carcinoma. 38
26813906 2016
31
Case Report: Bazex Syndrome Associated With Pulmonary Adenocarcinoma. 38
26765420 2016
32
Psoriasiform dermatitis and hepatocellular carcinoma: a new case of Bazex syndrome. 38
26333558 2015
33
Acute Onset of Acrokeratosis Paraneoplastica (Bazex Syndrome). 38
25760248 2015
34
Bazex syndrome (acrokeratosis paraneoplastica). 38
25065696 2014
35
A case of bazex syndrome with genital involvement. 38
25252025 2014
36
Bazex syndrome revealing a gastric cancer. 38
24926258 2014
37
[Hands cutaneous exam]. 38
24157184 2013
38
Bazex syndrome. 38
24346922 2013
39
Paraneoplastic cutaneous manifestations: concepts and updates. 38
23538999 2013
40
Re-exacerbation of cutaneous lesions in Bazex syndrome (acrokeratosis paraneoplastica) without tumor recurrence. 38
23220249 2012
41
Psoriasiform dermatitis in a case of newly diagnosed locally advanced pyriform sinus tumour: Bazex syndrome revisited. 38
22252191 2012
42
Multiple familial and pigmented basal cell carcinomas in early childhood - Bazex-Dupré-Christol syndrome. 38
21435027 2012
43
Coexistence of papuloerythroderma of Ofuji and acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of primary hepatocellular carcinoma. 38
22004495 2011
44
Cutaneous paraneoplasia. 38
21855730 2011
45
Acrokeratosis paraneoplastica (Bazex syndrome): report of a case associated with small cell lung carcinoma and review of the literature. 38
22470801 2011
46
Bazex syndrome (acrokeratosis paraneoplastica) diagnosed in a patient with oral persistent ulcerations. 38
20721648 2010
47
Chronic pruritus: a paraneoplastic sign. 38
21054705 2010
48
Diagnosis and treatment of cutaneous paraneoplastic disorders. 38
21054710 2010
49
Paraneoplastic syndromes in patients with primary oral cancers: a systematic review. 38
19833419 2010
50
Paraneoplastic acrokeratosis (bazex syndrome) in lung cancer. 38
19826120 2009
51
Three simultaneous paraneoplastic manifestations (ichthyosis acquisita, Bazex syndrome, and Leser-Trélat sign) with prostate adenocarcinoma. 38
19700026 2009
52
Acrokeratosis paraneoplastica (Bazex syndrome). 38
19439904 2009
53
Bazex syndrome (acrokeratosis paraneoplastica): persistence of cutaneous lesions after successful treatment of an associated oropharyngeal neoplasm. 38
18775590 2009
54
Acrokeratosis Paraneoplastica with Adenocarcinoma of the Colon Treated with Topical Tretinoin. 38
27303196 2008
55
Acrokeratosis paraneoplastica (Bazex' syndrome) associated with metastatic squamous cell esophageal carcinoma. 38
18838705 2008
56
Nevoid basal cell carcinoma syndrome (Gorlin syndrome). 38
19032739 2008
57
Acrokeratosis paraneoplastica Bazex syndrome associated with esophageal squamocellular carcinoma. 38
18672707 2008
58
Idiopathic basal cell carcinoma in children. 38
18505143 2008
59
Epidermal manifestations of internal malignancy. 38
18023768 2008
60
Basal cell carcinoma in childhood: case report and literature review. 38
17938034 2007
61
Paraneoplastic dermatosis suspected of being Bazex syndrome in a woman with breast cancer. 38
17951155 2007
62
Bazex syndrome or dermatomyositis? 38
17504722 2007
63
Acrokeratosis paraneoplastica (Bazex syndrome) presenting in a patient with metastatic breast carcinoma: possible etiologic role of zinc. 38
17374318 2007
64
Bazex syndrome (acrokeratosis paraneoplastica). 38
17292773 2007
65
[Pulmonary adenocarcinoma and Bazex syndrome (paraneoplastic acrokeratosis)]. 38
17257564 2007
66
[Paraneoplastic acrokeratosis (Bazex syndrome)]. 38
17185944 2006
67
[Acrokeratosis paraneoplastica with bullous lesions associated with esophageal squamous cell carcinoma]. 38
16796967 2006
68
[The syndrome of bazex or acrokeratosis paraneoplastica]. 38
17044630 2006
69
Acrokeratosis paraneoplastica (Bazex syndrome): case report and review of literature. 38
16638379 2006
70
Genetics of skin appendage neoplasms and related syndromes. 38
16272260 2005
71
Acrokeratosis paraneoplastica: Bazex syndrome. 38
15857461 2005
72
[Bazex syndrome, polymyalgia rheumatica or shoulder girdle syndrome?]. 38
15860179 2005
73
Acrokeratosis paraneoplastica (Bazex syndrome) preceeding the diagnosis of metastatic squamous cell carcinoma of the esophagus. 38
15793535 2005
74
Bazex syndrome (paraneoplastic acrokeratosis). 38
15605965 2004
75
Flexural Bazex syndrome associated with tonsillar adenocarcinoma. 38
15552609 2004
76
Acrokeratosis paraneoplastica (Bazex syndrome): an atypical presentation. 38
15347503 2004
77
Acrokeratosis paraneoplastica (Bazex syndrome) associated with breast cancer. 38
15245551 2004
78
Bullous lesions in Bazex syndrome and successful treatment with oral psoralen phototherapy. 38
11903162 2001
79
[Erythema and hand desquamation. Paraneoplastic acrokeratosis (Bazex syndrome)]. 38
11594141 2001
80
[Paraneoplastic Bazex acrokeratosis in adenocarcinoma of the stomach]. 38
11256024 2001
81
Bazex syndrome. 38
11455158 2001
82
Bazex syndrome: acrokeratosis paraneoplastica in association with simultaneous multiple genitourinary tumors. 38
10869650 2000
83
Acrokeratosis paraneoplastica (Bazex syndrome) with adenocarcinoma of the colon: report of a case and review of the literature. 38
10864355 2000
84
Bazex syndrome mimicking a primary autoimmune bullous disorder. 38
10321625 1999
85
Congenital ichthyosis, follicular atrophoderma, hypotrichosis, and hypohidrosis: a new genodermatosis? 38
9450882 1998
86
Bazex syndrome (acrokeratosis paraneoplastica). 38
9592679 1998
87
Acrokeratosis paraneoplastica: Bazex syndrome. 38
8949310 1996
88
Noninfectious hair disorders in children. 38
8954270 1996
89
Acrokeratosis paraneoplastica. 38
20947965 1996
90
Acrokeratosis paraneoplastica (Bazex syndrome) occurring with acquired ichthyosis in Hodgkin's disease. 38
7547410 1995
91
Acrokeratosis paraneoplastica (Bazex syndrome) associated with primary cutaneous squamous cell carcinoma of the lower leg, vitiligo and alopecia areata. 38
7669622 1995
92
Bazex syndrome: acrokeratosis paraneoplastica. 38
7640201 1995
93
Acrokeratosis paraneoplastica of Bazex in association with a metastatic neuroendocrine tumour. 38
7629771 1995
94
Bazex syndrome (acrokeratosis paraneoplastica). 38
7646396 1995
95
Acrokeratosis paraneoplastica (Bazex syndrome) with oropharyngeal squamous cell carcinoma. 38
7796616 1995
96
Familial syndromes with skin tumor markers. 38
7944188 1994
97
[Paraneoplastic acrokeratosis (Bazex' syndrome) associated with metastatic adenopathy of the neck from a differentiated carcinoma simultaneous with a well differentiated carcinoma of the oral cavity]. 38
7864310 1994
98
Bazex' syndrome. 38
8339198 1993
99
Bazex syndrome. 38
1408240 1992
100
Acrokeratosis paraneoplastica of Bazex: report of a case in a young black woman. 38
1521479 1992
101
Bazex syndrome (acrokeratosis paraneoplastica). An analytic review. 38
2067411 1991
102
[Skin manifestation of internal malignancy]. 38
2003738 1991
103
Psoriasiform dermatitis in a cachectic man. Acrokeratosis paraneoplastica (Bazex' syndrome). 38
3190261 1988
104
[Disorders of the skin appendages in Bazex syndrome]. 38
3254340 1988
105
Bazex syndrome with unusual clinical manifestations. 38
3220633 1988
106
[Paraneoplastic acrokeratosis: Bazex disease. A tumor-specific dermatosis in squamous cell cancers in the area of the head and neck]. 38
3410752 1988
107
[Useful cutaneous markers of internal malignancy in the early stage]. 38
3382228 1988
108
[Paraneoplastic acrokeratosis (Bazex syndrome)]. 38
2956219 1987
109
Acrokeratosis paraneoplastica (Bazex' syndrome). 38
3819049 1987
110
Paraneoplastic acrokeratosis: Bazex syndrome. 38
3720352 1986
111
Bazex syndrome. Follicular atrophoderma and basal cell epitheliomas. 38
4055198 1985
112
Acrokeratosis paraneoplastica (Bazex' syndrome). 38
6703754 1984
113
Acrokeratosis paraneoplastica (Bazex' syndrome). Report of a case and review of the literature. 38
6225397 1983
114
[X-chromosome-linked hereditary dermatoses]. 38
6804414 1982
115
The Bazex syndrome: follicular atrophoderma with multiple basal cell carcinomas, hypotrichosis and hypohidrosis. 38
7273474 1981
116
[A rare paraneoplastic syndrome: Bazex' syndrome (author's transl)]. 38
7332191 1981
117
[Bazex syndrome or acromegalic psoriasiform dermatosis]. 38
169676 1975
118
[The Bazex syndrome. A new para-neoplastic syndrome]. 38
5067168 1972

Variations for Bazex Syndrome

Cosmic variations for Bazex Syndrome:

9 (showing 1, show less)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM11166 TP53 skin,arm,other,keratosis c.406C>T p.Q136* 17:7675206-7675206 0

Expression for Bazex Syndrome

Search GEO for disease gene expression data for Bazex Syndrome.

Pathways for Bazex Syndrome

Pathways related to Bazex Syndrome according to GeneCards Suite gene sharing:

(showing 2, show less)
# Super pathways Score Top Affiliating Genes
1 10.65 TGFA FGF7
2 10.42 TGFA FGF7

GO Terms for Bazex Syndrome

Biological processes related to Bazex Syndrome according to GeneCards Suite gene sharing:

(showing 3, show less)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.16 TGFA FGF7
2 positive regulation of epithelial cell proliferation GO:0050679 8.96 TGFA FGF7
3 positive regulation of cell division GO:0051781 8.62 TGFA FGF7

Molecular functions related to Bazex Syndrome according to GeneCards Suite gene sharing:

(showing 1, show less)
# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 8.62 TGFA FGF7

Sources for Bazex Syndrome

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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