A Case of Elephantiasis Neuromatosa / 대한피부과학회지

ABSTRACT

We report herein a case of elephantiasis neuromatosa seen in neurofibromatosis patient, who was a 33-year-old male and had been diagnosed at the age of 13. Since that time on, small nodule present on the mid-back area led to develop grotesquely large, dark-brown colored, flabby mass with thick verucoid skin change, measuring about 30 * 20cm in size. Histopathologic examination of excised mass demonstrated moderate hyperplasia of epidermis and typical neurofibroma findings with soft tissue and fibrous tissue overgrowth, but no atypical cells. The electron microscopic study showed many perineurial cells surrounded by the continuous bssal lamina of fibroblsst in the dermal lesion and numerous giant pigmented granules in melanocyte and basal keratinocyte in the cafe-au-lait spot.