Egyptian Journal of Dermatology and Venerology

: 2020  |  Volume : 40  |  Issue : 1  |  Page : 66--67

Woolly hair: a rare disorder

Jyoti Budhwar, Chetna Singla 
 Department of Dermatology, Venereology and Leprosy, Sri Guru Ramdas Institute of Medical Sciences and Research, Vallah, Amritsar, Punjab, India

Correspondence Address:
Jyoti Budhwar
Assistant Professor Department of Skin & STD Sri Guru Ramdas Institute of Medical Science and Research, Vallah, Amritsar, 143001

How to cite this article:
Budhwar J, Singla C. Woolly hair: a rare disorder.Egypt J Dermatol Venerol 2020;40:66-67

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Budhwar J, Singla C. Woolly hair: a rare disorder. Egypt J Dermatol Venerol [serial online] 2020 [cited 2020 May 28 ];40:66-67
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Woolly hair is a rare congenital structural anomaly of scalp hair. It is either sporadic or genetic. Hair is extremely curly, and the texture resembles sheep’s wool, whereas the hair color often shows no unusual characteristics [1]. The rate of hair growth is usually normal, but the anagen phase is truncated, resulting in shorter hair. The hair shaft exhibits elliptical cross-section, axial rotation, and kink formation. The condition was first observed and described in a European family by Gossage [2]. It is different from the curly hair of black people, in that the curled hair of black people lies separately, whereas the curls of woolly hair usually merge.

A 10-year-old female child born of a nonconsanguineous marriage presented with complaints of short, curly brittle hair since birth ([Figure 1] and [Figure 2]). Similar complaints were also present in an 8-year-old female sibling ([Figure 3] and [Figure 4]). There were no similar complaints in parents and relatives. There was no history suggestive of physical and mental retardation. Examination of scalp revealed the presence of fine, short, coiled, and poorly pigmented hair. There were sparse body hair and partial loss of lateral third of eyebrows. Nails and teeth were normal. Systemic examination and routine hematological investigations were within the normal limits. Light microscopy of hair showed a slight variation in the thickness of the shaft, and potassium hydroxide examination result of hair was normal. Based on clinical findings and no systemic involvement, diagnosis of autosomal-recessive variant of woolly hair was made.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Woolly hair can appear as a part of systemic disease (woolly hair syndrome) or without systemic findings (nonsyndromic woolly hair). Nonsyndromic woolly hair can be inherited as either an autosomal dominant or recessive disorder. An earlier classification has described four types of woolly hair: hereditary woolly hair, familial woolly hair, symmetrical circumscribed allotrichia, and woolly hair nevus [3]. In the literature, woolly hair has been associated with ichthyosis, keratosis pilaris, deafness, acral keratoderma, nail changes, and dental and facial abnormalities [4],[5].

Two characteristic associations of woolly hair are Naxos disease and Carvajal disease. Naxos disease is characterized by woolly hair, palmoplantar keratoderma, and dilated cardiomyopathy with right ventricular dysplasia. It is an autosomal-recessive disorder occurring owing to mutation in the plakoglobin gene. Carvajal disease is similar clinically to Naxos disease, except for left ventricular involvement and presentation at a younger age, and it is due to mutation in the desmoplakin gene [6].

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