Korean J Dermatol.  2019 Jul;57(6):328-331.

Bilateral Segmental Neurofibromatosis with Lentiginosis Showing a Checkerboard Pattern

• Park SH
• Lee SH
• Kim SC

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.

Keyword

Body patterning; Lentiginosis; Neurofibromatosis

MeSH Terms

Axilla
Body Patterning
Buttocks
Dermis
Humans
Lentigo*
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1
Parturition
Skin