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CASE REPORT
Year : 1991  |  Volume : 57  |  Issue : 6  |  Page : 299-300

Atrichia congenita




Correspondence Address:
Y S Marfatia


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  Abstract 

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

How to cite this URL:
Marfatia Y S, Sankar R. Atrichia congenita. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2018 Oct 22];57:299-300. Available from: http://www.ijdvl.com/text.asp?1991/57/6/299/3713


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 [1], 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 [2], 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 [3] in 1964. Shy and Triestar observed a family with 6 of 13 siblings showing only alopecia (quoted by Muller. [4]).

Porter's cases [5], 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 Top


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 Top


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 Top

1.Pajtas J : Totale familiare heriditare Hypotrichose in 4 Generationen, Dermatologica, 1950 ; 101 ; 90-93.  Back to cited text no. 1    
2.Tillman W.G. : Alopecia congenita, Report of two families, Brit Med. J, 1952 : 2 : 428.  Back to cited text no. 2    
3.Linn H.W. Congenital atrichia, Aust J Dermatol 1964; 7 : 223-224.  Back to cited text no. 3    
4.Muller S A : Alopecia, Syndromes of Genetic Significance, J Invest Dermatol, 1973; 60:473-489.  Back to cited text no. 4    
5.Porter P S : Genetic disorders of hair growth, J Invest Dermatol, 1973 : 60 : 493-502  Back to cited text no. 5    


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[Figure - 1], [Figure - 2]



 

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