Nævus éruptifs sur épidermolyse bulleuse héréditaire

Abstract

EB naevus (EBN) are little-known, atypical, eruptive, pigmented melanocytic lesions that may occur in former sites of bullae occurring in epidermolysis bullosa hereditaria (EBH). We sought to describe the characteristics of such lesions and assess their course.This was a retrospective, two-centre study in which data was collated from the medical files of patients with EBN. We analyzed the patients' demographical data as well as the clinical, dermatoscopic, pathological features of EBN and their progression.Eight patients were studied: they were principally Caucasian (5/8), with a sex ratio of 1. All variants of EBH were represented and most were recessive (63%). We analysed 22 EBN, all atypical and emerging before the age of 10 years (73%), ubiquitously distributed and measuring greater than 5 cm(2) (25%). Of the 13 EBN subjected to dermatoscopy, 12 exhibited a benign reticular pattern. Four were biopsied, and analysis revealed three common naevi and one lentigo. After a median follow-up of 8 years, the EBN seen were either stable (68%), had regressed (23%) or had disappeared (one case). No cases of melanoma were diagnosed.EBN are acquired and atypical pigmented naevi. Sixty-four cases of EBN have been reported in the literature up to date. The dermatoscopic features may be evocative of melanoma (17/23 EBN), but to our knowledge no cases of melanoma at a naevus site have been reported. Recessive transmission of EBH appears to be a risk factor (63% of cases), a finding supported by certain pathophysiological hypotheses.EBN present atypical clinical and dermatoscopic features. However, while prophylactic total excision did not appear warranted in the absence of any reported cases of melanoma, regular clinical follow-up is recommended.