|Year : 1991 | Volume
| Issue : 6 | Page : 299-300
YS Marfatia, Rajesh Sankar
Y S Marfatia
Source of Support: None, Conflict of Interest: None
Three sisters aged 10, 13 and 14, presented with absence of scalp and facial hair. The youngest sister had scalp hair at birth, but had hair fall later, while the other two did not have hair at birth. Three other siblings had normal long hair, and there was no family history of any similar condition. Examination showed complete absence of scalp, axillary and pubic hair in all the three sisters.
Keywords: Atrichia congenita
|How to cite this article:|
Marfatia Y S, Sankar R. Atrichia congenita. Indian J Dermatol Venereol Leprol 1991;57:299-300
Atrichia congenita is the terminology used to refer to the group of patients, who presented with total alopecia at birth, and no other abnormality. Pajtas , in 1950 reported four generations of a family consisting of 147 members, 22 of whom had inherited alopecia universalis as an incompletely dominant trait, with similar numbers of each sex affected. Tillman , in 1952, reported two families with atrichia congenita inherited as an autosomal recessive trait. Two isolated cases of congenital alopecia, a boy and a girl, born of unrelated parents, were reported by Linn  in 1964. Shy and Triestar observed a family with 6 of 13 siblings showing only alopecia (quoted by Muller. ).
Porter's cases , also fit into this category. The autosomal recessive pattern of this disorder is characterised by total alopecia at birth, while in the autosomal dominant pattern, there is normal hair at childhood, which is shed from the first decade onwards. We are herewith reporting three siblings with atrichia congenita.
| Case Report|| |
Three sisters aged 14, 13 and 10, respectively came to the outpatient department of S.S.G.Hospital, Baroda, with absence of hair over head and eyebrows [Figure - 1]. The first two sisters did not have any hair at birth and did not have any hair since the, while the third one had scalp hair at birth, which fell off within two months. Even now, she has a single hair over the head and scattered hair over the eyebrows, which appear, grow and fall off within two months. There is no history of disturbances in dentition, or of epilepsy or mental retardation, or intolerance to heat, or of repeated respiratory infection. Patients do not have any associated skin or nail disorders. All the three children were delivered following prolonged labour. Milestones of development were normal, but none of the three have attained menarche.
These three children are the third, fourth and fifth among six daughters, the first, second and sixth having long scalp hair [Figure - 2]. They have no brothers. They were born to non-consanguineous parents, in whose families, there is no history of any similar disorder.
The subjects were well built for the ages, well oriented, with normal nails and teeth. There were no other skin lesions like eczema, palmoplantar keratoderma, keratin cysts of poikiloderma. They had total absence of terminal hair in the scalp, over the eyebrows and eyelashes [Figure - 1], over the axilla and pubes. Vellus hair was present all over the body. Histopathological examination showed normal epidermis with complete absence of hair follicles.
| Comments|| |
Atrichia congenita may be found associated with a variety of defects like keratin cysts, which are characterised by horny papules appearing initially over the face, neck and later extending to the trunk. Other associated defects that go alongwith atrichia congenita include mental retardation, epilepsy (Moynahan's Syndrome) or palmoplantar keratoderma (Hidrotic ectodermal dysplasia). It may also be seen as a solitary disorder.
Considering the age of onset of alopecia (i.e. since or soon after birth), and the absence of any nail, teeth or any other skin lesions, the diagnosis of atrichia congenita, as an isolated disorder was finalised. Patients were recommended to wear wigs, as not other treatment was feasible in these cases.
| References|| |
|1.||Pajtas J : Totale familiare heriditare Hypotrichose in 4 Generationen, Dermatologica, 1950 ; 101 ; 90-93. |
|2.||Tillman W.G. : Alopecia congenita, Report of two families, Brit Med. J, 1952 : 2 : 428. |
|3.||Linn H.W. Congenital atrichia, Aust J Dermatol 1964; 7 : 223-224. |
|4.||Muller S A : Alopecia, Syndromes of Genetic Significance, J Invest Dermatol, 1973; 60:473-489. |
|5.||Porter P S : Genetic disorders of hair growth, J Invest Dermatol, 1973 : 60 : 493-502 |
[Figure - 1], [Figure - 2]