Giant Congenital Melanocytic Nevus with Developmental Dysplasia of Bilateral Hip: A Rare Association
A 7-month-old male child presented to us with a large, pigmented flat lesion of irregular shape and colour, covering almost 70% of the trunk. The lesion was present since birth and has been following a proportional growth with the growth of the child. The mother also gave a history of noticing asymmetry of girth of the thighs since birth. The child has been otherwise healthy and there was no history of trauma or any neurological symptoms. He was delivered through a full term normal vaginal delivery without any obstetric complications. His physical, mental, and social milestones were appropriately achieved without any signs of developmental delay till the present date. The mother denied noticing any changes suggestive of malignant transformation like sudden accelerated growth, ulceration, bleeding, and induration in the nevus. There was no family history of similar presentations.\nOn cutaneous examination, the child had a large, irregular, well-defined, unevenly pigmented, flat melanocytic nevus with diffuse bluish-brown to black color, encircling the entire trunk. The maximum diameter was measured to be 33 cm, extending from the upper back to the left thigh, posteriorly. It had a smooth surface, with localized hypertrichosis over the left thigh. There were a total of 20 satellite lesions distributed over the arms, forearms, upper trunk, thighs, and legs, beyond the border of the main nevi. The child also had a swelling, measuring about 6 cm in diameter, on the lateral side of left lumbar area covered by the GCMN [].\nThe swelling was well defined, soft and non-tender, mobile, without any surface changes. Systemic examination was unremarkable. Palpation of spines revealed no abnormalities. The child had asymmetry of thighs’ girth, with the mid-thigh circumference of the right thigh measuring 4 cm more in contrast with the left thigh. Systemic examination was unremarkable. Mucosa, hair, nail, palms, and soles were normal. Ophthalmologic and neurological examinations revealed no abnormalities. On investigation, complete hemogram, biochemical parameters, and urine complete examination were all within normal limits. X-ray of hip showed developmental dysplasia of bilateral hip. Ultrasound examination of both hips demonstrated non-ossified femoral cartilage [].\nBrain and spinal magnetic resonance imaging was performed to detect neurocutaneous melanocytosis, which revealed a normal study. An excision biopsy from the nevus showed nests of rounded melanocytes in the papillary dermis extending deep into the reticular dermis confirming the diagnosis of CMN. Biopsy was performed from the mass over the left lumbar area, which revealed mature adipocytes, suggestive of lipoma [].