Dr. P. P. Mandal...... The Surgeon

Dr. P. P. Mandal, MS (General Surgery) General & Laparoscopic Surgeon Clinical Presentation An elderly woman presented with numerous, slowly progressive, painless cutaneous swellings distributed over the face, neck, upper limbs and trunk. Clinical examination revealed hundreds of soft, skin-coloured to hyperpigmented papules and nodules of varying sizes. Several lesions were pedunculated, while others were sessile. The morphology was highly characteristic of multiple cutaneous neurofibromas . A hyperpigmented café-au-lait macule was also noted over the trunk. The combination of extensive cutaneous neurofibromas and characteristic pigmentary change strongly suggests Neurofibromatosis Type 1 (NF1) . Figure 1.  Extensive cutaneous neurofibromas involving the face, neck and upper extremities. Figure 2.  Café-au-lait macule over the trunk in association with multiple cutaneous neurofibromas. Discussion Neurofibromatosis Type 1 is an autosomal dominant neurocutaneous disorder c...