Indian Journal of Dermatology, Venereology and Leprology Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL) ISSN: 0378-6323 EISSN: 0973-3922 Vol. 69, Num. 3, 2003, pp. 250-251

Indian Journal of Dermatology, Venereology & Leprology, Vol. 69, No. 3, May-June, 2003, pp. 250-251

Letter to Editor

Familial woolly hair in three generations

S. Chidambara Murthy

Address for correspondence: Dr. S. Chidambara Murthy, M. D., Department of Skin and Sexually Transmitted Diseases, S. N. Medical College and H. S. K. Hospital and Research Centre, Bagalkot-587101, Karnataka, India.

Code Number: dv03022

Sir,

I read the article "Familial woolly hair" by Prasad et al (Indian J Dermatol Venereol Leprol 2002;68:157) and wish to report a similar case, present in three generations of a family.

A 5-year-old non-atopic boy born of a consanguineous marriage was referred by the Pediatrics Department for evaluation of abnormal hair over the scalp since birth. There were no delayed milestones, physical or mental retardation or photosensitivity. Examination revealed short, tightly coiled, thin, dry, poorly pigmented, brittle hair over the scalp. The eyebrows were sparse but the eyelashes were normal. The palms and soles were not involved and the nails, teeth and genitalia were normal. His systemic examination was normal. There was no ocular or skeletal involvement. The patient's family pedigree showed similar involvement in three generations. There was inbreeding within the family.

Routine hematological and urinary examinations were normal. Blood VDRL, liver function tests, blood urea, serum creatinine, and blood sugar were normal. Light microscopic examination of the hair was normal. Electron microscopic examination could not be done for the want of this facility.

Woolly hair refers to tightly coiled hair covering the whole scalp or part of it, in a non-negroid individual.¹ Four types have been described:² 1) Hereditary woolly hair, 2) Familial woolly hair, 3) Symmetrical circumscribed allotrichia, and 4) Woolly hair nevus. Woolly hair in association with keratosis pilaris atrophicans and cataract,¹ keratosis pilaris and curling of eyelashes,³ palmoplantar keratoderma and cardiac involvement⁴ have been reported.

Inbreeding within the family, presence of woolly hair in children born consanguineously, and the absence of this abnormality in the parents of affected children, suggestive of autosomal recessive inheritance, were points favoring the diagnosis of familial woolly hair. However, there were no associated cutaneous or systemic abnormalities in them. Cases of familial woolly hair are rarely reported and its occurrence in three generations of a family is still rarer.

References

1. Neild VS, Pegum JS, Wells RS. The association of keratosis pilaris atrophicans and woolly hair with and without Noonan's syndrome. Br J Dermatol 1984;110:357-61.
2. Hutchison PE, Cairns RJ, Wells RS. Woolly hair. Trans St John's Hosp Dermatol Soc 1974;60:160-76.
3. Thappa DM, Thadeus J, Garg BR. Woolly hair. Indian J Dermatol 1995;40:181-5.
4. Barker JNWN, Protonotorios N, Tsatopoulou A, et al. Palmoplantar keratoderma, curly hair and endomyocardial fibrodysplasia: A new syndrome. Br J Dermatol 1983;119(suppl 33):13-4.

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