A Case of Woolly Hair

Woolly hair is characterized by fair, fine hair. It is found most frequently in Negroes, but is unusual in people of non-Negroid origin. Woolly hair is present at birth and is usually most severe during childhood, when it is often impossible to brush the hair. Adult life brings variable amelioration of the condition.

The growth rate is probably normal, but the hair may not grow longer than a few centimeters, perhaps because the growth cycle is shortened. In the familial group, the hairs on the arms, legs, pubic and axillary regions and eyebrows may be short and pale. There are no associated cutaneous or systemic diseases. Hutchinson et al. classified woolly hair into three groups: (1) hereditary woolly hair, which is typically inherited as an autosomal dominant trait; (2) familial or sporadic woolly hair, probably inherited as an autosomal recessive trait; and (3) woolly hair nevus, a nonhereditary, partial scalp involvement by woolly hair of a markedly reduced diameter. The former two types are characterized by generalized scalp involve­ment. A fourth group was described by Ormerod et al, who reported a family with short, fine, kinky hair interspersed with normal hair throughout their scalp. We think our case can be classified as sporadic woolly hair, because there were no other woolly hair cases in her family, she had a sporadic occurrence and there were distinctive clinical findings. cialis super active

Woolly hair has been reported in association with palmoplantar keratoderma and cardiac abnormali­ties. The triad of woolly hair, palmoplantar kerato­derma and right ventricular cardiomyopathy defines Naxos disease, which in at least some cases may be caused by mutations in the desmosomal protein, plakoglobin. Mutation in another desmosomal pro­tein, desmoplakin, has been implicated in the patho­genesis of woolly hair with palmoplantar kerato­derma and dilated cardiomyopathy, also called Car­vajal syndrome. Both Naxos disease and Carvajal syndrome are inherited in an autosomal recessive fashion, whereas most other cardiomyopathies are inherited through autosomal dominant transmi­ssion. Of these conditions, electrocardiographic cha­nges, arrhythmias, heart failure, and even sudden cardiac death have been reported. Therefore the presence of woolly hair with palmoplantar keratoder­ma should trigger further evaluation for cardiac disease. Skin fragility/woolly hair syndrome, usually classified among the epidermolysis bullosa disorder, is an autosomal recessive disorder that, like Carvajal syndrome, exhibits woolly hair, palmoplantar kerato­derma and mutation in the desmoplakin gene. Car­diac findings, however, are not characteristic of skin fragility/woolly hair syndrome.

Several other conditions such as curly hair-ankylo- blepharon-nail syndrome and tricho-dento-osseous syndrome have been associated with very curly hair that clinically may be difficult to distinguish from true woolly hair. canadian cialis