Woolly hair in two siblings.

Sir,

Hair disorders present clinically in varied forms, one of the rare entities being the group of woolly hair disorders.[1] The main clinical types are hereditary woolly hair, familial woolly hair, symmetrical circumscribed allotrichia, and woolly hair nevus.[2]

A 5-year-old girl and her 7-year-old brother presented with abnormal hair on scalp since birth. There was no history of parental consanguinity. The two younger siblings had normal hair and there was no other systemic illness in them. Parents gave the history that scalp hair was sparse since birth and progressively coiled as the children grew older. On examination, scalp hair were scanty, extremely curly, coarse-textured, and evenly pigmented [Figures 1 and 2]. There was no palmoplantar keratoderma and electrocardiography and echocardiography were normal. Rest of the systemic examination revealed no abnormality. All the milestones achieved were appropriate for age. All the routine hematological investigations were within the normal limits.

Figure 1

Sparse, coarse textured, curly hair on the scalp of the 5-year-old girl

Figure 2

Woolly hair in two siblings

Woolly hair syndrome is a group of disorders involving structural defects in hair shaft without increase in the fragility. Familial woolly hair is probably transmitted as an autosomal recessive inheritance and there is reduction in the diameter of hair shafts, which may be poorly pigmented. The hair is brittle and on light microscopy, and the hair shaft is oval with a significantly reduced diameter. Scanning electron microscopy shows signs of cuticle wear and tear.[3] Few cases of woolly hair have been reported in non-negroid races.