1/3. Partial unilateral lentiginosis with ipsilateral Lisch nodules and axillary freckling.A 9-year-old Taiwanese girl had an unusual combination of multiple lentigines clustered on her left face, 3 cafe-au-lait spots on her left arm and ipsilateral axillary freckling. Two Lisch nodules of the iris were recognized in the left eye but none in the right eye, which is why we established a diagnosis of segmental neurofibromatosis 1. No family member had any sign of neurofibromatosis 1. Moreover, the patient had a large speckled lentiginous nevus involving the left side of her trunk, and her father showed a similar skin disorder on his left buttock. These lesions were taken to be unrelated to the patient's segmental neurofibromatosis 1. This case provides further evidence in favor of the concept that partial unilateral lentiginosis represents a mosaic manifestation of type 1 neurofibromatosis.- - - - - - - - - - ranking = 1keywords = lentiginosis (Clic here for more details about this article) |
2/3. Multiple lentigines syndrome (leopard syndrome or progressive cardiomyopathic lentiginosis).The multiple lentigines syndrome is an autosomal dominant condition which has many similarities to noonan syndrome, except in the most striking feature from which its name is derived. The less neutral but very apt mnemonic, leopard syndrome, was first used by Gorlin et al to whom the major debt in the definition of this syndrome lies, that is, Lentigines, ECG abnormalities, Ocular hypertelorism/Obstructive cardiomyopathy, pulmonary valve stenosis, Abnormalities of genitalia in males, Retardation of growth, and deafness. Not previously included in the mnemonic is cardiomyopathy which is an important feature because it is associated with significant mortality.- - - - - - - - - - ranking = 0.8keywords = lentiginosis (Clic here for more details about this article) |
3/3. Naevi spili, cafe-au-lait spots and melanocytic naevi aggregated alongside Blaschko's lines, with a review of segmental melanocytic lesions.This is the first case in the literature describing naevi spili, cafe-au-lait spots and melanocytic naevi aggregated along-side Blaschko's lines. The pattern of melanocytic lesions in our patient is different from the congenital pigmentary syndromes and the segmental distribution of melanocytic naevi, the quadrant distribution of dysplastic naevi or the partial unilateral lentiginosis which here are shortly reviewed. The distribution may be a result of a somatic mutation occurring at an early stage of embryogenesis when neural structures had already been formed.- - - - - - - - - - ranking = 0.2keywords = lentiginosis (Clic here for more details about this article) |