Speckled lentiginous nevus syndrome with median nerve paresis: A rare syndrome with a new association
A 23-year-old Indian girl presented with a large pigmented lesion on her left arm and hand since birth. She reported darkening of the hyperpigmented patch over the years with the development of darkly pigmented spots over it. She also complained of weakness of the grip of left hand for the past 3 years. There was no history of trauma. Her medical history was otherwise unremarkable.\nCutaneous examination revealed a large café au lait macule running along the antero-medial aspect of left arm extending down to involve the palmar and the dorsal surface of the hand []. This macule was superimposed by multiple dark brown macules of size 0.2-1 cm. There was mild atrophy of the thenar eminence of the left hand []. Motor examination revealed deficient opposition and thumb flexion with positive pen test. Sensory examination revealed sensory loss to touch, temperature, and pain over medial nerve distribution of hand. A complete neurological checkup did not reveal any additional findings.\nNerve conduction studies showed involvement of the left median nerve (both motor and sensory) as non-recordable with normal left ulnar nerve and right median (both motor and sensory) nerve conduction. There was weakness in the left abductor pollicis brevis muscle on electromyography documentation. Magnetic resonance imaging (MRI) of the cervical spine was normal. Histopathological examination from one of the darker macule showed features consistent with nevus spilus [].