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Basan syndrome

MedGen UID:
140808
Concept ID:
C0406707
Disease or Syndrome
Synonyms: Absence of dermatoglyphics congenital milia; Absence of fingerprints congenital milia; Absence of fingerprints-congenital milia syndrome; Baird syndrome; Ectodermal dysplasia absent dermatoglyphics; ECTODERMAL DYSPLASIA, ABSENT DERMATOGLYPHIC PATTERN, CHANGES IN NAILS, AND SIMIAN CREASE; Ectodermal dysplasia, absent dermatoglyphic pattern, with change in nails and simian crease
SNOMED CT: Basan syndrome (239011004)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): SMARCAD1 (4q22.3)
 
Monarch Initiative: MONDO:0007507
OMIM®: 129200
Orphanet: ORPHA1658

Definition

Complete congenital absence of dermatoglyphs is a rare syndrome characterized by autosomal dominant inheritance of the lack of ridges on palms and soles, neonatal acral blisters and facial milia, adult traumatic blistering and fissuring, absent or reduced sweating of palms and soles, and contracture of digits. Additional features may include single palmar transverse crease, palmoplantar keratoderma, and nail grooving (summary by Limova et al., 1993). [from OMIM]

Clinical features

From HPO
Single transverse palmar crease
MedGen UID:
96108
Concept ID:
C0424731
Finding
The distal and proximal transverse palmar creases are merged into a single transverse palmar crease.
Tapered finger
MedGen UID:
98098
Concept ID:
C0426886
Finding
The gradual reduction in girth of the finger from proximal to distal.
Cutaneous syndactyly of toes
MedGen UID:
320423
Concept ID:
C1834737
Congenital Abnormality
A soft tissue continuity in the anteroposterior axis between adjacent foot digits that involves at least half of the proximodistal length of one of the two involved digits; or, a soft tissue continuity in the A/P axis between two digits of the foot that does not meet the prior objective criteria.
Palmoplantar keratoderma
MedGen UID:
1635750
Concept ID:
C4551675
Disease or Syndrome
Abnormal thickening of the skin of the palms of the hands and the soles of the feet.
Flexion contracture of digit
MedGen UID:
326595
Concept ID:
C1839864
Anatomical Abnormality
A bent (flexed) finger or toe joint that cannot be straightened actively or passively. It is thus a chronic loss of joint motion due to structural changes in muscle, tendons, ligaments, or skin that prevents normal movement of joints.
Ectodermal dysplasia
MedGen UID:
8544
Concept ID:
C0013575
Disease or Syndrome
Ectodermal dysplasia is a group of conditions in which there is abnormal development of the skin, hair, nails, teeth, or sweat glands.
Nail dystrophy
MedGen UID:
66368
Concept ID:
C0221260
Disease or Syndrome
Onychodystrophy (nail dystrophy) refers to nail changes apart from changes of the color (nail dyschromia) and involves partial or complete disruption of the various keratinous layers of the nail plate.
Epidermal acanthosis
MedGen UID:
65136
Concept ID:
C0221270
Finding
Diffuse hypertrophy or thickening of the stratum spinosum of the epidermis (prickle cell layer of the skin).
Knuckle pads
MedGen UID:
78103
Concept ID:
C0264000
Disease or Syndrome
Skoog (1948) defined knuckle pads as 'subcutaneous nodules on the dorsal aspect of the proximal interphalangeal joints.'
Milia
MedGen UID:
87528
Concept ID:
C0345996
Anatomical Abnormality
Presence of multiple small cysts containing keratin (skin protein) and presenting as tiny pearly-white bumps just under the surface of the skin.
Hypermelanotic macule
MedGen UID:
375013
Concept ID:
C1842774
Finding
A hyperpigmented circumscribed area of change in normal skin color without elevation or depression of any size.
Adermatoglyphia
MedGen UID:
338875
Concept ID:
C1852150
Finding
Isolated congenital adermatoglyphia is a rare, genetic developmental defect during embryogenesis disorder characterized by the lack of epidermal ridges on the palms and soles, resulting in the absence of fingerprints, with no other associated manifestations. It is associated with a reduced number of sweat gland openings and reduced transpiration of palms and soles.
Acral blistering
MedGen UID:
1375545
Concept ID:
C4476927
Finding
Bullae (defined as fluid-filled blisters more than 5 mm in diameter with thin walls) of the skin with an acral distribution (affecting peripheral regions such as hands and feet).
Palmoplantar hypohidrosis
MedGen UID:
1678196
Concept ID:
C5194130
Disease or Syndrome
Decreased sweating on the palms and soles.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBasan syndrome
Follow this link to review classifications for Basan syndrome in Orphanet.

Recent clinical studies

Etiology

Luna PC, Larralde M
Pediatr Dermatol 2012 Jul-Aug;29(4):527-9. Epub 2011 Sep 9 doi: 10.1111/j.1525-1470.2011.01473.x. PMID: 21906143

Diagnosis

Xiong Y, Chen T, Yu J, Zhou H, Lu B, Chen L, Sun L, Wang C, Li S, Wu B
Dis Markers 2022;2022:7840710. Epub 2022 May 10 doi: 10.1155/2022/7840710. PMID: 35592705Free PMC Article
Nieto-Benito LM, Molina-López I, Feito-Rodríguez M, Martínez-González V, Suárez-Fernández R, Campos-Dominguez M
Pediatr Dermatol 2021 Mar;38(2):530-532. Epub 2021 Jan 24 doi: 10.1111/pde.14512. PMID: 33486784
Li M, Wang J, Li Z, Zhang J, Ni C, Cheng R, Yao Z
Eur J Hum Genet 2016 Aug;24(9):1367-70. Epub 2016 Mar 2 doi: 10.1038/ejhg.2016.15. PMID: 26932190Free PMC Article
Marks KC, Banks WR 3rd, Cunningham D, Witman PM, Herman GE
Am J Med Genet A 2014 May;164A(5):1188-91. Epub 2014 Mar 24 doi: 10.1002/ajmg.a.36438. PMID: 24664640

Prognosis

Loh AYT, Špoljar S, Neo GYW, Escande-Beillard N, Leushacke M, Luijten MNH, Venkatesh B, Bonnard C, van Steensel MAM, Hamm H, Carmichael A, Rajan N, Carney TJ, Reversade B
Am J Med Genet A 2022 Jun;188(6):1752-1760. Epub 2022 Feb 25 doi: 10.1002/ajmg.a.62703. PMID: 35212137

Clinical prediction guides

Loh AYT, Špoljar S, Neo GYW, Escande-Beillard N, Leushacke M, Luijten MNH, Venkatesh B, Bonnard C, van Steensel MAM, Hamm H, Carmichael A, Rajan N, Carney TJ, Reversade B
Am J Med Genet A 2022 Jun;188(6):1752-1760. Epub 2022 Feb 25 doi: 10.1002/ajmg.a.62703. PMID: 35212137
Li M, Wang J, Li Z, Zhang J, Ni C, Cheng R, Yao Z
Eur J Hum Genet 2016 Aug;24(9):1367-70. Epub 2016 Mar 2 doi: 10.1038/ejhg.2016.15. PMID: 26932190Free PMC Article