On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.1 Rio de Janeiro Jan./Feb. 2004
Woolly hair nevus syndrome*
Josenildo Rodrigues de OliveiraI; Viviane Tom MazoccoI; Lúcia Helena F. de ArrudaII
Doctor, Department of Dermatology at the Faculdade de Medicina de Jundiaí
IIAssistant Professor, Department of Dermatology at the Faculdade de Medicina de Jundiaí
Woolly Hair Nevus is a rare, nonhereditary condition characterized by tightly curled hair localized on one or several areas of the scalp. The authors present a case of woolly hair nevus with onset during childhood, which is associated with palmoplantar keratoderma and cardiopathy. It is named woolly hair nevus syndrome.
Key-words: heart diseases; keratosis.
Wooly hair nevus is a rare, nonhereditary condition characterized by an anomaly in the growth of hair. Its onset is usually during the first years of life, although there have been cases described as occurring during adolescence. It affects both sexes alike. The affected hairs are lighter, shiny, with smaller diameter and waves. They show normal growth and are not fragile. The disorder occurs in a circumscribed area of the scalp, which may be one or many in number.1-7
In association with changes on the scalp, the presence of the following has been recorded: pigmented or epidermal nevus, verrucuous nevus, palmoplantar keratodermia, ocular abnormalities,11 in addition to cardiomiopathies with premature cardiac insufficiency.1,8,9,12,13
Hutchinson et al.13 have classified woolly hair into three groups: (I) hereditary, which is typically inherited in an autosomal dominant pattern; (II) familiar, probably inherited in an autosomal recessive pattern; (III) woolly hair nevus, nonhereditary with localized involvement on the scalp. The first two are characterized by a generalized involvement of the scalp. Post14 divides woolly hair nevus into three subtypes: type 1 - without any cutaneous involvement; type 2 - accompanied by the linear nevus of the skin; type 3 - acquired in adult life, the scalp shows features of pubic hair.2,3,5,7
The authors present a case of woolly hair nevus with onset during childhood, in association with palmoplanter keratodermia and cardiopathy. It includes the aforementioned woolly hair nevus syndrome, which is a term proposed by Al Harmozi et al.4
In the present review, Souza et al.15 describe the case of this one-year-old male patient, who showed alteration of hair color, texture and form in association with epidermal nevus.
A six-year old Caucasian-Mongoloid male child, born and raised in Jundiai, showed alterations in the color, texture and form of the hairs in various areas of the body. The alteration began at the age of one in well-delimited areas (left frontal, right parietal and occipital regions). His mother referred to the altered hairs as being lighter in color than the others.
At three years of age the patient showed thickening of the bilateral palmar and left plantar regions as well as brown spots over his body, mainly on the trunk.
Psychomotor development was normal. There was no consanguinity or similar cases in the patient's family history.
The dermatological examination verified: (1) circumscribed areas of the light-brown hairs that were fine, shiny and tightly curled on the frontal right and left parietal regions, on the vertex and occipital region (Figures 1 and 2); (2) keratotic plaques on the palmar and marginal regions of the left foot (Figure 3); (3) brown spots with a linear disposition affecting the anterior side of the left superior member and thorax; (4) brown spots linearly disposed on the hyoid and posterior cervical regions (Figure 4); (5) keratotic and hyperchromic plaques on the back of the left foot (Figure 5). There were no ocular or ungueal alterations.
Laboratory tests (hemogram, serum biochemistry, X-rays of the thorax and two-dimensional echocardiogram with Doppler) showed no alterations. The electrocardiogram detected a conduction disturbance in the right branch of the His bundle.
The case reported in this paper represents woolly hair nevus Post type 2,14 in which, apart from the alterations limited to the scalp, an association of epidermal nevus and verrucous nevus occurs in 50 percent of cases. Also observed were palmoplantar keratodermia and cardiac conduction disturbance. These additional findings have led Al Harmozi4 to propose the expression 'Woolly Hair Nevus Syndrome', covering woolly hair nevus and other alterations. Four previous reports exist on its association with palmoplantar keratodermia, tightly curled hair and cardiac abnormalities.
In 1988, the first association of cardiac abnormality was detected in an electrocardiographic study of two child patients of 9 and 12 years of age. After four years of follow-up, the patients evidenced dilated echocardiographic cardiomiopathy and later death by cardiac failure.1 Protonotarios et al.9 describe four families of seven afflicted persons showing diffuse palmoplantar keratoderma, tightly curled hair and severe arrhythmogenic right ventricular dysplasia. Barker et al.12 report on 17 patients with woolly hair, palmoplantar keratodermia with an erythematous border associated with the dilated and hypokinetic right ventricle. Tosti et al.8 describe two patients of the same family who have tightly curled hair with diffuse palmoplantar keratodermia of the Thost-Unna type, in association with asymptomatic ventricular disorder. Huerta et al.1 studied 18 patients who were woolly hair carriers, each of whom had palmoplantar keratodermia in association with cardiac abnormality.
This report, like the case described by Protonotarios et al.,9 Barker et al.,12 Tosti et al.,8 Huerta et al.1 suggest that the association of any keratodermia with woolly hair may be complicated by potentially lethal cardiac disorders on the right or left sides, which much be investigated. The first cardiac abnormality is manifested in an electrocardiographic disturbance;1 therefore the patient remains in cardiological follow-up in order to prematurely detect any echocardiographic abnormalities.
For this reason, the authors recommend a cardiological evaluation of all patients showing an association of woolly hair nevus with palmoplantar keratodermia.
This case is particularly important, because Souza et al.15 described it as woolly hair nevus and epidermal nevus in this same dermatological review back in 1998. The child was one year old. Today, she is six, and shows palmoplantar keratodermia and electrocardiographic alterations (disturbance of conduction in the His bundle). This demonstrates that these cases must be rigorously followed up in view of preventing its progression into cardiomiopathy, and thereby reducing morbid mortality.
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in December, 20th of 2002
Approved by the Consultive Council and accepted for publication in January, 10th of 2003
* Work done al "Serviço de Dermatologia do Hospital das Clínicas de Franco da Rocha, Faculdade de Medicina de Jundiaí".