Published: 2022-01-24

Twisted tressess: a rare case series of familial woolly hair

Masarat Jabeen, Gurkim Kaur, Ennakshee Sharma, Devraj Dogra


Woolly hair is an uncommon congenital anomaly of the scalp hair presenting with strongly coiled hair involving a localized area of the scalp or covering the entire side and occurring in non-black people. Among the syndromes presenting with woolly hair, the most known are the Naxos syndrome, the Carvajal-Huerta syndrome, the woolly hair/hypotrichosis, the ectodermal dysplasia-skin fragility and the tricho-hepato-enteric syndrome. Case characteristics: herein, we report a case of autosomal recessive familial woolly hair of an Asian female child and member of a family, her younger brother affected by a localised woolly hair, not associated to complications. Children with woolly hair must be examined completely and monitored regularly to rule out cardiofaciocutaneous and Noonan syndrome.


Kinky hair, Light microscopy, Naxos syndrome, Carvajal syndrome, Woolly hair

Full Text:



Gossage AM. The inheritance of certain human abnormalities. Quartz J Med. 1907;1:331-47.

Hutchinson PE, Cairns RJ, Wells RS. Woolly hair. Clinical and general aspects. Trans St Johns Hosp Dermatol Soc. 1974;60:160-77.

Vasudevan B, Verma R, Pragasam V, Badad A. A rare case of woolly hair with unusual associations. Indian Dermatol Online J. 2013;4:222-4.

Ramot Y, Zlotogorski A. The twisting tale of woolly hair: a trait with many causes. J Med Genet. 2015;52:217-23.

Naveen KN, Shetty SR, Radha H, Athaniker SB, Gundannanavar MR. Woolly hair with trichoscopic features. Indian J Paediatr Dermatol. 2016;17:56-7.

Singh A, Mathur D, Yadav S, Nijhawan M, Aggarwal P, Soni S et al. Woolly hair a rare presentation. Journal of Evolution of Medical and Dental Sciences. 2013;2:10051-55.

Singh SK, Manchanda K, Kumar A, Verma A. Familial Woolly Hair: A Rare Entity. Int J Trichology. 2012;4:288-89.

Matsuno N, Kunisada M, Kanki H, Simomura Y, Nishigori C. A Case of Autosomal Recessive Woolly Hair/Hypotrichosis with Alternation in Severity: Deterioration and Improvement with Age. Case Rep Dermatol. 2013;5(3):363-67.

Chien AJ, Valentine MC, Sybert VP. Hereditary woolly hair and keratosis pilaris. J Am Acad Dermatol. 2006;54:S35-9.