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Familial Woolly Hair: A Rare Entity

International Journal of Trichology
Medknow Publications
Publication Date
DOI: 10.4103/0974-7753.111214
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Sir, Woolly hair is a rare congenital abnormality of the structure of the scalp hair characterized by tightly coiled hair involving part or the entire scalp occurring in an individual of non-negroid origin.[1] It was first observed and described by Gossage in 1907 in a European family.[2] In 1974, Hutchinson et al. classified woolly hair into three variants: A localized variant (woolly hair nevus) and two generalized variants, including autosomal dominant hereditary woolly hair and autosomal recessive (AR) familial woolly hair.[3] In addition to these, diffuse partial woolly hair has also been described as a separate entity.[4] We hereby describe a family with AR familial woolly hair. A 15-year-old female born of a non-consanguineous marriage presented with complaints of short, curly, brittle hair since birth. There was no history suggestive of physical or mental retardation and no history suggestive of systemic involvement. Similar history was also present in three of her siblings, i.e. an 18-year-old male, a 13-year-old female, and a 5-year-old male. There was no history of similar complaints in the parents and the two other siblings. Examination revealed the presence of fine, short, coiled, dry poorly pigmented brittle hair over the scalp [Figure 1]. There were sparse body and pubic hair, partial loss of the lateral third of eyebrows, and absence of axillary hair. Nail and teeth were normal. All the affected members were examined and had similar findings. Systemic examination was within normal limits in all the affected family members. Routine hematological investigations including complete blood counts, liver and renal function tests, chest radiograph, and urinalysis were within the normal limits. Light microscopic examination of the hair was normal. Potassium hydroxide (KOH) examination of the hair revealed no fungal growth. Detailed cardiac evaluation including electrocardiogram and echocardiography was normal. Genetic analysis to determine the gene loci could not be carried out due to the non-

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