Verrucous epidermal naevus associated with woolly hair naeves

Shaikh Manzoor, Naseer Ahmed Mir, Seema Qayoom

 Department of Dermatology, STD and Leprosy and Dept. of Orthopaedics, Government SKIMS Medical College, Hospital Bermina, Srinagar, Kashmir, India

Correspondence Address:
Shaikh Manzoor
C/o. Empire Medicate, Opposite Fire Service Head Quarters, Batamalla, Srinagar (J&K) - 190 001
India

Introduction

Epidermal naevi are hamartomatous lesions arising from the embryonic ectoderm. The pluri-potential ectodermal cells evolve into a variety of differentiated cell types, including keratinocytes and the cells forming the various epidermal appendages. Epidermal naevi are conveniently sub divided according to their predominant cell type, though this tends to be some what artificial as such naevi often compromise more than one epidermal cell types.[1]

In some cases, most commonly when there are multiple lesions, there may be associated defects in other tissues, particularly the skeleton and the CNS, here the term epidermal naevus syndrome is often applied.[2]

Woolly hair is more or less tightly coiled hair occurring over the entire scalp or part of it, in an individual not of Negroid origin.[3] There is much confusion over the classification of various woolly hair types. Hutchinson and co-workers have proposed following classification.[4]

1. Hereditary woolly hair, 2. Familial woolly hair, 3. Symmetrical circumscribed allotrichia, 4. Woolly hair naevus.

We report an unusual case of verrucous epidermal naevus associated with features of symmetrical circumscribed allotrichia, and woolly hair noevus.

Case Report

A 25-year-old male, product of a non consanguinous marriage reported to us with the history of nonpruritic thickened linear lesion over the neck of 20 years duration. Besides he had noticed thinning of his hairs on the right side of the scalp starting from frontal side and extending upto occiput. There was retarded growth of hairs in this area. No family history of such problem was present. His general physical examination was normal. Nails and mucous membranes were normal. Local examination revealed a verrucous slightly hyperpig-mented linear noevus on the right side of chest. Besides this, on right side of the scalp, hairs were slightly thinner, and had retarded growth, extending from fronto-parietal area upto the occiput. The skin underlying this area was normal. Hairs on rest of the scalp and body were normal. Routine investigations on blood and urine were normal. Histological examination of naevus revealed hyperkeratosis, and papill-omatbsis. Acanthosis was seen at places.

Light microscopy of the hair did not reveal any abnormality. Electron microscopy was not atte-mpted.

On the basis of clinical and histological evidence a diagnosis of verrucous epidermal naevus associated with woolly hair naevus was made.

Discussion

Epidermal naevi are hamartomatous lesions arising from the embryonic ectoderm. Blaschkos documented that linear streaks and swirls adopted by these naevi may be due to somatic mutation, each lesion comprising the progeny of a single mutant keratinocyte. A genetic influence on the development of epidermal naevi is suggested by occasional reports of their familial origin.[6] Wide spread multiple epidermal naevi may reflect genetic or chromosomal mosaicism.[7] There is strong support for such a view, at least in the case of those epidermal naevi that demonstrate the histological features of epidermolytic hyperkeratosis, since there have now been several reports of affected individuals having children with classical bullous ichthyosis.[8] Verrucous epidermal naevi may be associated with defects in the other tissues particularly the skeleton and CNS, and the term epidermal naevus syndrome is often applied in such cases.[9]

Megalopinna has been reported in a case with epidermal naevus.[10] Verrucous epidermal, naevus has been reported in association with scalp woolly hair naevi.[11] Woolly hair is more or less tightly coiled hair occurring over the entire scalp or part of it, in an individual not of Negroid origin.[3] There is confusion over its nomenclature and only widely accepted classification is given by Hutchinson and co-workers.

In our patient verrucous epidermal naevus was associated with a woolly hair naevus, consisting of coarse hairs which were remarkably shorter in size extending from fronto-parietal region up to occipital region, thus had features both of symmetrical circumscribed allotrichia and woolly hair naevus. A similar case was reported by Bovenmyer,[12] without associated verrucous epidermal naevus.

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