ABSTRACT
Agminated lentiginosis (AL) is a rare pigmented disorder that is characterized by numerous lentigines in a unilateral distribution or often in a segmental pattern corresponding to one or more dermatomes. AL coexists with other several diseases and some researchers have suggested it is a variant of dermatomal neurofibromatosis if AL is accompanied by cafe-au-lait (CAL) macules or neurofibromas. We report here on a case of a 12-year-old female who presented with multiple lentigines on her right neck and shoulder (the C2 and C3 dermatomes) combined with CAL macule and ipsilateral axillary freckling. On checking the family history, her mother had grouped lentigines on her right chest (the T4, T5 dermatomes). But there were no neurofibromas, Lisch nodules and neurologic or skeletal abnormalities in both of them.