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Familial woolly hair
G K Prasad
Department of Dermatology, Base Hospital, Delhi Cantt -10
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Prasad G K. Familial woolly hair. Indian J Dermatol Venereol Leprol 2002;68:157
AbstractWoolly hair is tightly coiled hair occurring over the entire scalp or part of it, in an individual of non- Negroid origin. Two such cases occurring in a family is reported due to its rarity.
Wide spectrum of hair disorders present clinically in varied forms, one of the rare entities being the group of wooly hair disorders. 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.
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.
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. 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.
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