|Year : 2016 | Volume
| Issue : 3 | Page : 232-234
Congenital bilateral symmetric Becker's nevus with neurological involvement
Minu Jose Chiramel1, Roshni Menon1, Brinda G David1, Sandhya Panjeta Gulia2
1 Department of Dermatology, Venereology and Leprosy, Sri Venkateshwaraa Medical College Hospital and Research Centre, Ariyur, Puducherry, India
2 Department of Pathology, Sri Venkateshwaraa Medical College Hospital and Research Centre, Ariyur, Puducherry, India
|Date of Web Publication||5-Jul-2016|
Minu Jose Chiramel
Department of Dermatology, Venereology and Leprosy, Sri Venkateshwaraa Medical College Hospital and Research Centre, Ariyur - 605 012, Puducherry
Source of Support: None, Conflict of Interest: None
Becker's nevus is an epidermal nevus presenting as hyperpigmented macules. Onset in infancy, bilateral distribution, location on forearms, and associated neurological manifestations makes this case of Becker's nevus worth reporting.
Keywords: Becker's, bilateral, congenital
|How to cite this article:|
Chiramel MJ, Menon R, David BG, Gulia SP. Congenital bilateral symmetric Becker's nevus with neurological involvement. Indian J Paediatr Dermatol 2016;17:232-4
|How to cite this URL:|
Chiramel MJ, Menon R, David BG, Gulia SP. Congenital bilateral symmetric Becker's nevus with neurological involvement. Indian J Paediatr Dermatol [serial online] 2016 [cited 2021 May 10];17:232-4. Available from: https://www.ijpd.in/text.asp?2016/17/3/232/179504
| Introduction|| |
Becker's nevus is a common epidermal nevus which is characterized by hyperpigmented macules with hypertrichosis and an onset in adolescence. It is rarely multiple, rarely congenital in onset, and sometimes associated with systemic features. We present to you a case of Becker's nevus, which is both bilateral and of early onset along with neurological dysfunction.
| Case Report|| |
A 13-year-old boy presented to our outpatient department with complaints of hyperpigmented macules on bilateral forearms since infancy. The lesions were noticed by the parents at birth, were light brown to start with and became darker and slightly larger with age. There was increased hair density on these lesions. There was no history of acneiform papules, underlying plaques or bony defects. However, the child had delayed milestones, difficulty in speech, and spastic paraparesis since infancy. The child was confined to a wheelchair and unable to carry out daily activities without support. There was a history of urinary incontinence.
The child was a product of nonconsanguineous marriage, born at home by normal vaginal delivery. There was a history of prolonged labor with probable birth asphyxia which required neonatal Intensive Care Unit care.
On examination, there were dark brown large macules on extensor aspect of both forearms about 10–15 cm in size with ill-defined geographic, splashed borders. The lesion on the right forearm was also extended to the adjacent upper arm [Figure 1]. There was hypertrichosis overlying these macules [Figure 2]. Apart from these lesions, there was a single café au lait macule of 3–4 cm size over the lower abdomen. The child had an abnormal facies with protruding eyes, hypertelorism, and flattened nasal bridge. He was unable to follow commands. Neurological examination showed atrophy of upper limb and lower limb muscles, probably secondary to disuse. There was spasticity of upper limbs and lower limbs with power of 3/5 in lower limbs and near normal power in upper limbs. Deep tendon reflexes were exaggerated.
|Figure 1: Both forearms show large dark brown macules on extensor aspects with splashed borders|
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With this clinical picture, the first diagnosis considered was bilaterally symmetric Becker's nevus with neurological involvement. The neurological features were either associated hypoxic ischemic encephalopathy and cerebral palsy or as a direct association with Becker's nevus. Other differential considered was pigmented hypertrichosis with insulin dependent diabetes syndrome though the site of lesions, absence of binding down, and absence of clinical evidence of diabetes mellitus ruled it out.
On skin biopsy, epidermis showed hyperkeratosis with mild acanthosis and squaring of rete pegs. There was increased basal layer pigmentation seen. These findings were diagnostic of Becker's nevus [Figure 3]. In other investigations, a computed tomography scan of the brain was performed to rule out any organic lesions, but no abnormality was found. There were no ocular or auditory deficiencies.
|Figure 3: Photomicrograph of lesion on left forearm showing mild acanthosis, squaring of rete pegs, and increased basal layer melanization suggestive of Becker's nevus (H and E, ×10)|
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With clinical and histological evidence, the diagnosis of bilateral, symmetric Becker's nevus of congenital onset was confirmed. The associated neurological features and dysmorphic facies are probably part of Becker's nevus syndrome, but a chance association of cerebral palsy cannot be completely ruled out.
| Discussion|| |
Becker's nevus, otherwise known as pigmented hairy epidermal nevus, has a prevalence of around 0.5% and is more common in males. Shoulders, upper chest, and back are the common sites of involvement. It usually presents as dark brown hyperpigmented macules with a splashed tomato appearance of borders. There is associated hypertrichosis in around 50%. It usually starts around adolescence, becomes darker and larger over few years and tends to persist. Being an organoid hamartomatous nevus, it may be associated with underlying smooth muscle hamartomas. Acneiform papules may develop on it as a result of increased susceptibility to androgens. The term Becker's nevus syndrome refers to the presence of ipsilateral breast hypoplasia, cutaneous, or musculoskeletal anomalies in association with Becker's nevus. Rarer associations of Becker's nevus include localized lipoatrophy, spina bifida, scoliosis pectus carinatum, congenital adrenal hyperplasia, and accessory scrotum. It is known to be associated with neurofibromatosis and café au lait macules.
On histopathology, Becker's nevus has both epidermal and dermal changes. Epidermis shows regular acanthosis with squaring of rete pegs and increased basal layer pigmentation. Dermis shows few melanophages, smooth muscle hyperplasia, and increased number of sweat glands and sebaceous glands.
There are multiple reports of Becker's nevus occurring on atypical sites and of early onset. In our case, the presence of bilaterality, onset since infancy, and the associated café au lait macule warranted a special attention. Our patient also had some neurological features which may be a part of Becker's nevus syndrome or a chance association secondary to the birth asphyxia. The presence of slightly dysmorphic facies with hypertelorism and a depressed nasal bridge made us suspect the former, but certainty of causation cannot be attained.
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]