Widow’s peak

Widow's peak is a morphogenetic trait that has been present since birth and is thought to be inherited in an autosomal dominant manner. Although in most cases it is a normal variant, a number of authors have pointed out that this trait is often the result of a slight hypertelorism due to a lateral displacement of the hair growth suppression zones around the orbit. The tapered hairline must be distinguished from age-related migration of the hairline (maturing hairline), which can also result in a median tapered hairline.

The pointed hairline trait is inherited in an autosomal dominant manner. The "tapered hairline" allele dominates over the "straight hairline" allele.

The "pointed hairline" is characterized by a descending V-shaped area in the middle of the frontal hairline (see Fig.). A classification system based on the height and width of a pointed hairline has not yet been described. A pointed hairline is merely an indicative morphological feature for numerous syndromes (see table), but not a criterion for a specific syndrome.

The English name Widow's peak is probably a reference to the shape of a cap (biquoquet) worn by widows in the 16th century. In the English-speaking world, this term is used for both sexes. It used to be believed that the lengthening of the front hairline predicted a woman's early widowhood (Kyriakou G et al. 2021). From the 16th to the mid-19th century, English widows wore a pointed mourning hood after the death of their husband. It is believed that this costume dates back to the Queen Anne de Bretagne, who wore a striking black V-shaped hood after the death of her husband, King Charles VIII (Allanson JE et al. (2009)

In the following syndromes, which are also associated with hypertelorism, the "pointed hairline" phenotype is also frequently developed:

• Aarskog-Scott syndrome/FGD1 gene/X-linkedrecessive/OMIM:305400
• Aarskog-Scott syndrome/gene unknown/ autosomal dominant/OMIM:100050)
• Acrofacial dysostosis, Catania type/gene unknown/autosomal dominant/OMIM:101805
• Acro-fronto-facio-nasal dysostosis type 1,2 /gene unknown/ autosomal recessive/ OMIMJ:201180-239710
• Congenital glycosylation disorder type IIIc/SLC53C1 gene/autosomal recessive/OMIM:266265
• Distal trisomy 17q /gene unknown/ORPHA 3379
• Facial neuromuscular syndrome, Cyprus type/gene unknown/autosomal dominant/ORPHA:3379
• Cranio-fronto-nasal dysplasia (CFNS)/EFNB1 gene/X-linked dominant/OMIM:304110
• Distal monosomy 10q/gene unknown/OMIM:609625-96148
• Donnai-Barrow syndrome/LRP2 gene/autosomal recessive/OMIM:222448,2143
• Facio-digito-genital syndrome/gene unknown/autosomal recessive/OMIM: 227330
• Fronto-nasal dysplasia 1/ALX3 gene/autosomal recessive/OMIM:136s760
• Fronto-nasal dysplasia , severe microphthalmia-facial cleft syndrome /ALX1 gene/autosomal recessive/OMIM:613456,306452
• Frontorhinia/ALX3 gene/autosomal recessive/OMIM:236760,391474
• Hypertelorism and other facial dysmorphism, brachydactyly, genital anomalies, mental retardation, gene unknown/autosomal recessive/ OMIM:614684
• Infantile hypotonia-oculomotor anomalies, hyperkinetic movement disorders, developmental delay syndrome/SYT1 gene/mode of concealment unknown/OMIM:618218-ORPHA:522077

• Epidermolysis bullosa dystrophica recessive, generalized intermediate (historical: non-Hallopeau-Siemens)/ LAMC2 gene/ OMIM: 226650

• Mental retardation, microcephaly, growth retardation, joint contractures, facial dysmorphism /gene unknown/autosomal recessive/OMIM:606242
• Nasopalpebral lipoma-coloboma syndrome/ZDBF2 syndrome/OMIM:167730-ORPHA:2399
• NEDBEH syndrome (developmental disorder of the nervous system)/RERE gene/autosomal dominant/OMIM:616975,ORPHA:494344
• Opitz syndrome/MID1 gene/ X-linked recessive/OMIM:300000; ORPHA 2745
• Opitz-GG/BBB syndrome/SPECC1L gene/ autosomal dominant/OMIM:145410, ORPHA:306588
• Opitz-Kaveggia syndrome/MED12 gene/X-linked/ OMIM:145410; ORPHA 306558
• Sweeney-Cox syndrome/TWIST1 gene/autosomal dominant(OMIMM:617746
• Teebi hypertelorism syndrome/SPECC1L gene/autosomal dominant/OMIM:145420;ORPHA:1519. A second Teebi phenotype is associated with mutations in the CDH11 gene.
• Waardenburg syndrome (WS1)/PAX3 gene- gene locus 2q35/autosomal dominant/OMIM:193500;
• Widow`s peak syndrome/gene:unknown/ X-linked dominant/OMIM: 314570
• X-linked intellectual disability, Stoll type/gene_unknown/X-linked recessive/ORPHA:85326
• Zimmermann-Laband syndrome/ATP6V1B2 gene/autosomal dominant/OMIN:626355; ORPHA:3473