Bilateral Segmental Neurofibromatosis with Lentiginosis Showing a Checkerboard Pattern
- Author:
Seh Hyun PARK
1
;
Si Hyung LEE
;
Soo Chan KIM
Author Information
1. Department of Dermatology, Gangnam Severance Hospital, Seoul, Korea. kimsc@yuhs.ac
- Publication Type:Case Report
- Keywords:
Body patterning;
Lentiginosis;
Neurofibromatosis
- MeSH:
Axilla;
Body Patterning;
Buttocks;
Dermis;
Humans;
Lentigo;
Neurofibroma;
Neurofibromatoses;
Neurofibromatosis 1;
Parturition;
Skin
- From:Korean Journal of Dermatology
2019;57(6):328-331
- CountryRepublic of Korea
- Language:English
-
Abstract:
Segmental neurofibromatosis, a subtype of neurofibromatosis type 1, is characterized by neurofibromas and/or café-au-lait spots limited to an area or segment of the body. Checkerboard pattern is a rare type of cutaneous mosaic manifestation, characterized by squares or broad ribbons of affected skin with sharp demarcation at the midline. Herein, we report the case of a patient with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern. Our patient had multiple hyperpigmented macules on her entire body in a checkerboard pattern since birth. Several café-au-lait patches were observed on the left buttock and right axilla. A neurofibroma was incidentally found beneath the café-au-lait patch by histological examination, which showed ill-defined spindle cells with elongated nuclei at the deep dermis that stained positive for S-100. Based on the clinical presentation and histopathologic results, the patient was diagnosed with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern.
