Total Alopecia (ATRICHIA CONGENITA)

As an isolated abnormality

Total alopecia as an apparently isolated defect is usually determined by an autosomal recessive gene. Some pedigrees have been traced back to the early l9th century . Dominant or irregular dominant inheritance has occurred in some families. The two genotypes seem to be phenotypically indistinguishable, but detailed investigation would probably reveal differences. The term 'total' is relative, but if any hairs are present they are extremely few. Many isolated cases and families reported under the diagnosis of congenital alopecia are found on review of the original reports to be unquestionably examples of other syndromes; many were hidrotic ectodermal dysplasia.

Pathology: The hair follicles are absent in adult life, even when the fetal hair-coat has been normal. Sebaceous glands are smaller than normal. When a few stray hairs have survived the structure of the shaft appears to be normal.

Clinical features: The scalp hair is often normal at birth but is shed between the first and sixth months, after which no further growth occurs. In some cases the scalp has been totally hairless at birth and has remained so. Eyebrows, eyelashes and body hair may also be absent, but more often there are a few straggling pubic and axillary hairs and scanty eyebrows and eyelashes. Teeth and nails are normal and general health, intelligence and expectation of life are unimpaired.

With associated defects

Total or almost total alopecia is unusual in hereditary syndromes.

Progeria: Scalp and body hair is totally deficient. Hydrotic eclodermal dysplasia. Total or almost total alopecia is associated with palmoplantar keratoderma and thickened discoloured nails. Any hairs that are present are structurally normal but are often finer than the average.

Moynahan's syndrome: This autosomal recessive syndrome, reported in male siblings, is associated with mental retardation, epilepsy and total baldness of the scalp; the hair may regrow in childhood between 2 and 4 years of age.

Atrichia with keratin cysts: This rare syndrome, comparable with the condition found in certain hairless mice, has been reported only in girls, but the mode of inheritance is unknown. Total and permanent alopecia develops after the first hair-coat is shed. At any age between 5 and 18, numerous small, horny papules appear, first on the face, neck and scalp, and then gradually over the greater part of the limbs and trunk. Histologically, the papules are thiek-walled keratin cysts. Baraitser's syndrome. This autosomaJ recessive syndrome presents as almost total alopecia following the loss of some downy scalp hair present at birth.

Three cases are reported in an inbred family: all had almost total alopecia of all sites, including eyebrows and lashes. There were occasional isolated hairs. Mental and physical retardation were associated.

Homoeopathic Remedies

There is no know cure for total alopecia. Constitutional and Miasmatic medicines like Baryta Carb, Bacillinum. Carcinosin, Thuja & vibronic remedy Brain are worth trying. As this is not a common phenomena most of the doctors never come across such cases. It is given here for the academic purpose only.

References

1. Baraitser M, Carter C, Brett EM. A new alopecia/mental retardation syndrome. J Med Genet 1983; 20: 64-75.
2. Birke G. Uber Atrichia congenita und Erbgang. Arch Dermatol Syphilol 1954; 197: 322-5.
3. Calvo Melendro J. Atriquia Congenita totalty permanente. Med Clin 1955; 24: 253-7.
4. Friederich HC. Zur Kenntnis der Kongenitale Hypertrichosis. Dermatol Wochenschr 1950; i21: 408-10.
5. Linn HW. Congenital atrichia. Aust J Dermatol 1964; 7: 223-5.
6. Moynahan EJ. Familial congenital alopecia. Proc Roy Soc Med 1962; SS: 411-12.
7. Rook AJ, Dawber RPR, eds. Diseases of the Hair and Scalp 2nd edn. Oxford: Blackwell Scientific Publications, 1991: 170-2.
8. Tillman WG. Alopecia congenita. Br Med J 1952; ii: 428-9.

01 Feb 2010