| Descriptor English: | Focal Facial Dermal Dysplasias | ||||||
| Descriptor Spanish: |
Displasias Dérmicas Faciales Focales
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| Descriptor Portuguese: | Displasias Dérmicas Faciais Focais | ||||||
| Descriptor French: | Dysplasies dermiques faciales focales | ||||||
| Entry term(s): |
Bitemporal Aplasia Cutis Congenita Bitemporal Forceps Marks Syndrome Brauer Setleis Syndrome Brauer Syndrome Brauer-Setleis Syndrome Dysplasia, Facial Ectodermal Ectodermal Dysplasia, Facial Ectodermal Dysplasias, Facial FFDD, Type 1 FFDD, Type 2 FFDD, Type 3 FFDD, Type 4 Facial Ectodermal Dysplasia Facial Ectodermal Dysplasias Focal Facial Dermal Dysplasia 1 Focal Facial Dermal Dysplasia 2 Focal Facial Dermal Dysplasia 3 Focal Facial Dermal Dysplasia 4 Focal Facial Dermal Dysplasia Type 1 Focal Facial Dermal Dysplasia Type 2 Focal Facial Dermal Dysplasia Type 4 Focal Facial Dermal Dysplasia, Type II Hereditary Symmetrical Aplastic Nevi of Temples Setleis Syndrome Syndrome, Brauer Type 1 FFDD Type 2 FFDD Type 3 FFDD Type 4 FFDD Type 4 FFDDs |
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| Tree number(s): |
C16.131.077.350.568 C16.131.831.350.568 C16.320.850.250.568 C17.800.804.350.568 C17.800.827.250.568 |
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| RDF Unique Identifier: | https://id.nlm.nih.gov/mesh/D000090303 | ||||||
| Scope note: | A heterogenous group of genetic disorders characterized by scar-like atrophic lesions on the temple region of the head including preauricular area. Location of skin defects is likely related to defects in fusion of embryonic facial prominences during development of the face. Focal facial dermal dysplasia (FFDD) is generally divided into four subtypes according to the location of the lesions and inheritance pattern: FFDD1 (Brauer syndrome); FFDD2 (Brauer-Setleis syndrome); FFDD3 (Setleis syndrome); and FFDD4. Mutations in TWIST2 Protein and/or CYP26C1 (see CYP26 FAMILY) are associated with FFDD3, and 4. |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| Previous Indexing: |
Ectodermal Dysplasia (1979-2021) |
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| Public MeSH Note: | 2022 |
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| History Note: | 2022 |
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| DeCS ID: | 59791 | ||||||
| Unique ID: | D000090303 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 2022/01/01 | ||||||
| Date of Entry: | 2021/07/09 | ||||||
| Revision Date: | 2021/05/10 |
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Focal Facial Dermal Dysplasias
- Preferred
Focal Facial Dermal Dysplasia 3
- Narrower
Focal Facial Dermal Dysplasia 1
- Narrower
Focal Facial Dermal Dysplasia 2
- Narrower
Focal Facial Dermal Dysplasia 4
- Narrower
| Concept UI |
M000746045 |
| Scope note | A heterogenous group of genetic disorders characterized by scar-like atrophic lesions on the temple region of the head including preauricular area. Location of skin defects is likely related to defects in fusion of embryonic facial prominences during development of the face. Focal facial dermal dysplasia (FFDD) is generally divided into four subtypes according to the location of the lesions and inheritance pattern: FFDD1 (Brauer syndrome); FFDD2 (Brauer-Setleis syndrome); FFDD3 (Setleis syndrome); and FFDD4. Mutations in TWIST2 Protein and/or CYP26C1 (see CYP26 FAMILY) are associated with FFDD3, and 4. |
| Preferred term | Focal Facial Dermal Dysplasias |
| Entry term(s) |
Dysplasia, Facial Ectodermal Ectodermal Dysplasia, Facial Ectodermal Dysplasias, Facial Facial Ectodermal Dysplasia Facial Ectodermal Dysplasias |
| Concept UI |
M000746067 |
| Preferred term | Focal Facial Dermal Dysplasia 3 |
| Entry term(s) |
FFDD, Type 3 Setleis Syndrome Type 3 FFDD |
| Concept UI |
M000746065 |
| Preferred term | Focal Facial Dermal Dysplasia 1 |
| Entry term(s) |
Bitemporal Aplasia Cutis Congenita Brauer Syndrome FFDD, Type 1 Focal Facial Dermal Dysplasia Type 1 Hereditary Symmetrical Aplastic Nevi of Temples Syndrome, Brauer Type 1 FFDD |
| Concept UI |
M000746066 |
| Preferred term | Focal Facial Dermal Dysplasia 2 |
| Entry term(s) |
Bitemporal Forceps Marks Syndrome Brauer Setleis Syndrome Brauer-Setleis Syndrome FFDD, Type 2 Focal Facial Dermal Dysplasia Type 2 Focal Facial Dermal Dysplasia, Type II Type 2 FFDD |
| Concept UI |
M000746068 |
| Preferred term | Focal Facial Dermal Dysplasia 4 |
| Entry term(s) |
FFDD, Type 4 Focal Facial Dermal Dysplasia Type 4 Type 4 FFDD Type 4 FFDDs |
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