Pastrana AE, Morales OD, García RMT

Language: English
References: 13
Page: 296-299
PDF size: 178.83 Kb.

ABSTRACT

Introduction: ungual alterations are extremely frequent in patients with epidermolysis bullosa (EB) and may even be associated with prognosis and outcomes. However, there are few studies characterizing nail involvement in EB.
Methods: we performed an observational cross-sectional descriptive study of children aged zero to 18 years with the diagnosis of EB cared for at the National Institute for Pediatrics, a tertiary referral center in Mexico City.
Results: we searched for nail involvement in 22 patients with EB (14 with recessive dystrophic EB, four with dominant dystrophic EB, two with EB simplex, one with junctional EB and one with Kindler syndrome) that attended our specialized multidisciplinary clinic. Patients had a median age of 9.2 years (range 0.4-17.5), and all had nail involvement. The most frequent nail changes across all subtypes were: anonychia (81.8%), hypotrophic/dystrophic nails (31.8%), pachyonychia (22.7%) and longitudinal ridging (4.5%).
Discussion/conclusion: we found all patients had nail alterations regardless of EB subtypes. Most of those with severe subtypes had anonychia at a young age. This descriptive study confirms nail changes are extremely frequent in all subtypes of EB. Further studies to characterize nail involvement in specific subtypes of EB and association with outcomes are needed.

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