Résumé
Giant congenital nevomelanocytic nevus (GCNN) is a rare variant of congenital melanocytic nevus measuring >20 cm in size that often has a garment-like distribution. Regular follow up is recommended because of a risk of melanoma transformation of 4.6%. We report a 14-year-old boy with gradual regression of giant congenital melanocytic nevus over the left upper limb, chest, back and axilla, whom we have followed-up since birth. At birth, a hyperpigmented jet-black patch without hair was present over the left side of torso and upper limb including palms and nails. Follow up at the ages of 1, 5, 11 and 14 years showed progressive spontaneous regression of the nevus resulting in shiny atrophic skin, diffuse hypopigmentation, lentigo-like macules, nodules and arthrogryphosis of affected areas. Histopathology of the lesions on follow-up revealed absence of pigmented nevus cells in the regressing areas and thickened sclerotic collagen bundles.
Sujets)
Adolescent , Arthrogrypose/anatomopathologie , Biopsie , Évolution de la maladie , Humains , Lentigo/anatomopathologie , Mâle , Naevus pigmentaire/congénital , Naevus pigmentaire/anatomopathologie , Rémission spontanée , Indice de gravité de la maladie , Peau/anatomopathologie , Tumeurs cutanées/congénital , Tumeurs cutanées/anatomopathologieRésumé
Giant congenital melanocytic nevus (garment nevus) is a kind of congenital malformation of neural crest cells with size greater than 20 cm. Malignant melanoma may develop in 2-31% of these lesions. The objective of this paper is to present a unique case of giant nevus which is rare.