Familial woolly hair

GK Prasad

 Department of Dermatology, Base Hospital, Delhi Cantt -10, India

Correspondence Address:
G K Prasad
Department of Dermatology, Base Hospital, Delhi Cantt -10
India

Introduction

Wide spectrum of hair disorders present clinically in varied forms, one of the rare entities being the group of wooly hair disorders.[1] The hair defect is structural without any increase in fragility. The main clinical types are hereditary woolly hair, familial woolly hair, symmetrical circumscribed allotrichia, and woolly hair nevus.

Case Report

A 16-year-old girl and her 12-year-old sister presented with abnormal hairs on the scalp since infancy. The elder sibs were mentally retarded. There′s no history of patchy loss of hair, photosensitivity, eye or bony abnormality. There was no history of any systemic illness. According to history given by the parents, hair growth was normal at birth. Progressive coiling of hair was noticed, as they grew older. There was parental history of consanguinity.

Examination revealed tightly coiled, thin dry, poorly pigmented, brittle hairs over the scalp [Figure - 1] and [Figure - 2]. Eyebrows and axillary hairs were coarse in both sisters. Nails, teeth and genitals were normal. There was no ocular or skeletal abnormality. Elder sister had a low IQ.

Microscopic examination of hair was essentially normal. Haematological and biochemical parameters were also normal.

Discussion

Woolly hair syndrome is a group of disorders involving structural defects in hair shaft without any increase in fragility. Familial woolly hair is probably transmitted as an autosomal recessive inheritance and there is a marked reduction in the diameter of hair shafts, which may be poorly pigmented. The hair is brittle and on light microscopy, hair shaft is oval with significantly reduced diameter. Scanning electron microscopy, shows signs of cuticle wear and tear.[2] Very few cases have been reported in non-negroid races and these two sisters are typical cases of familial woolly hair. In some patients, woolly hair may become darker and less curly with time, but there is no effective treatment.[3]

1.	Neild VS, Pegum JS, Wells RS. The association of keratosis pilaris atrophicans and woolly hair, with and without Nethertor's syndrome. Br J Dermatol 1984,110:357-361. [Google Scholar]
2.	Hutchison PE, Cairns RJ, Wells RS. Woolly hair. Trans St John's Hosp Dermatol Soc 1974;60:160-176. [Google Scholar]
3.	Rook A, Dawber R. Woolly hair, In: Diseases of Hair and Scalp, Boston, Mass. Blackwell Scientific Publications Inc. 1982:212-216. [Google Scholar]