A Case of Neurofibromatosis with Multiple Pseudoatrophic Macules

Nam, Kyung Hwa; Song, Ki Hun; Cho, Yong Sun; Rhee, Chin Ho; Yun, Seok Kweon; Ihm, Chull Wan; Kim, Han Uk

Abstract

Neurofibroma usually manifests as a soft nodule that protrudes from the skin surface of patients with neurofibromatosis. Pseudoatrophic macule is a rare variant of neurofibroma, and it is described as a slightly depressed macular lesion. To the best of our knowledge, only one case of neurofibromatosis associated with pseudoatrophic macules has been reported in the Korean dermatologic literature. A 20-year-old man presented with multiple round shaped macules scattered on the trunk. The surface of the skin lesion was slightly depressed compared with the normal adjacent skin. He had multiple cafe-au-lait macules on the body and freckles in the axillae and inguinal areas. Histopathologic examination of the depressed macule demonstrated a relatively well-demarcated mass that was composed of spindle cells intermingled with loosely arranged collagen fibers in the dermis. The spindle cells showed a positive reaction for S-100 protein. Herein, we report on a rare case of type 1 neurofibromatosis with multiple pseudoatrophic macules.