Correspondence Address:
Meghana Phiske
From the Department of Dermatology, Mahatma Gandhi Medical College, Navi Mumbai, Maharashtra
India

Full Text



Sir,

Verrucous epidermal nevus (VEN) presents with skin-coloured or brown verrucous papules and plaques, commonly located on the head, trunk, or extremities, with epidermal hyperplasia on histopathology. Woolly hair is a rare congenital structural abnormality of scalp hair without increased fragility, characterised by tightly coiled hair involving a part of the scalp or the entire scalp, occurring in non-Negroid individuals. Woolly hair nevus (WHN) is a localised, sporadic variant of woolly hair which is characterised by tightly curled unruly hair in the circumscribed area of scalp in non-black persons, appearing when scalp hair begins to grow. It presents as one or more patches of woolly hair which is smaller in diameter and lighter in colour than normal hair, in an otherwise normal field. WHN is frequently found in association with linear VEN of focal or systematized distribution noted in 50% of cases.[1] A unique association of ipsilateral VEN of focal distribution with WHN is highlighted.

A 24-year-old male born of non-consanguineous marriage came with a complaint of asymptomatic dark raised lesions over the left side of the nape of the neck and shoulder since birth. He had no systemic complaints or co-morbidities, and none of the family members were affected similarly. He had not taken any treatment for the same. Cutaneous examination revealed localised, well-defined hyper-pigmented linear and whorled verrucous plaques on the left side of the nape of the neck and shoulder not crossing the mid-line. The occipital and parietal region of the scalp showed two well-circumscribed patches of woolly hair, 3 × 3 cm on the ipsilateral side of nevus [Figure 1]a, [Figure 1]b, [Figure 1]c. Dermoscopy of the VEN showed multiple light-brown globules with an overlying trail of fine white scales with a few hairs [Figure 2]. The hair mount from WHN showed normally pigmented, thin curved hair [Figure 3]. Histopathology of the verrucous papule showed marked hyperkeratosis, parakeratosis, hypergranulosis, irregular psoriasiform hyperplasia, papillomatosis, and an increased pigment in the basal layer with perivascular lymphocytes in the upper dermis suggestive of VEN [Figure 4]a, [Figure 4]b. Systemic evaluation was normal. Routine blood and the biochemistry profile with radiological investigation were normal. The final diagnosis based on clinical, dermoscopic, and histopathologic evaluation was ipsilateral VEN with WHN without systemic associations.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Epidermal nevi are nests of epidermal cells which are noted at birth or within the first year as a thin plaque or linear tan patch often along Blaschko lines[2] and have multiple clinical variants including a verrucous type. VEN occurs as a result of hamartomatous proliferation of the keratinocytes. It has been hypothesised that VEN is a mosaic disorder, resulting from post-zygotic mutations affecting the keratin 1 (K1) and/or keratin 10 (K10) gene(s). VEN typically appears as verrucous, skin-coloured to brown papules or well-demarcated papillomatous plaques. They can be located anywhere, including the head, trunk, or extremities.[3] They are often present at birth as flat tan or brown marks, but they become more apparent in adulthood when they become thickened and often warty. They can be localised or systematised (unilateral or bilateral).[4] Rarely these lesions may be dermatomal in distribution. The diagnosis is largely clinical, aided by histopathology and dermoscopy. Dermoscopy is a widely used non-invasive diagnostic tool in the diagnosis of many dermatological diseases. Omar et al. described ten more non-vascular dermoscopic features of VEN [Table 1] apart from large brown circles, which are seen in the majority of VEN. We observed brown globules, fine scales, and hair, which were consistent with the findings of Omar et al.,[5] who concluded that dermoscopic examination of VEN may lead to more reliable clinical interpretation, thus reducing the need for histopathology. Histology, which is a gold standard for diagnosis of VEN, shows hyperkeratosis, acanthosis, and papillomatosis.[6]{Table 1}

Treatment of VEN is challenging since treatment modalities are often associated with recurrences, whereas more aggressive treatments often end up with scarring. Multiple therapeutic options include tretinoin (given in our patient with good response over 2 months) and 5-fluorouracil, acitretin, calcipotriol, dithranol, podophyllin, dermabrasion, or cryotherapy. Recently, CO2, argon, and pulsed dye lasers have emerged as promising treatment options, although long-term follow-up shows a high recurrence rate and several side effects such as hypo-pigmentation and scarring.[6]

VEN can be an isolated finding, or it can show syndromic or non-syndromic association with other cutaneous/extracutaneous lesions.[7] The localised variant of woolly hair, that is, WHN, is frequently associated with linear VEN of focal or systematised distribution.[8]

Woolly hair is a rare condition characterised by coiled, curly, and slightly hypo-pigmented hair involving a part of the scalp or the entire scalp occurring in a non-Negroid individual with an unknown etiopathogenesis. The affected hair has a smaller diameter but without increased fragility. It is not a form of hypotrichosis. Three main groups are described; two are diffuse and inherited (autosomal dominant), and one is localised and sporadic, known as WHN.[9] WHN, first described by Wise, refers to woolly hair restricted to a limited area of the scalp.[9] It appears when scalp hair begins to grow and arises from the scalp as one or more patches of woolly hair in an otherwise normal field. The hair is usually smaller in diameter and lighter in colour than the rest of the hair. Post-suggested three types of WHN: 1) in the young without any other nevoid changes in the scalp or glabrous skin, 2) in the young along with linear epidermal nevus, and 3) acquired in adult life.[10] Several associations of WHN are described, such as ophthalmologic, auditory, renal, cutaneous, dental, and skeletal manifestations.[11] The diagnosis is suspected clinically and can be confirmed on light and or scanning electron microscopy. Till date, there is no treatment for WHN except for surgery.[12] There is an association of ipsilateral VEN in half of patients with WHN.[1]

Both VEN and WHN can be isolated or associated with cutaneous or extra-cutaneous lesions, requiring a multi-disciplinary approach and evaluation. Unique association of VEN and WHN in a young male without any systemic association is highlighted.

Acknowledgement

Dr. Surina Nair, JR3, Department of Dermatology, Mahatma Gandhi Medical College, Navi Mumbai, for her contribution in dermoscopy of the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References