Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features

Congenital erosive and vesicular dermatosis (CEVD) healing with reticulated supple scarring, a condition usually observed in premature neonates, presents at birth with vesicles and erosions. Lesions typically heal within a few months, leaving behind scarring with a distinctive supple and reticulated texture.

We sought to merge existing literature with new cases to further define CEVD.

We analyzed 19 previous reports of CEVD and added 9 additional patients; we identified unifying characteristics of this cohort.

In 28 total cases, notable features included: preterm birth (79%), nail abnormalities (46%), hyperthermia/hypohidrosis (46%), a history of maternal chorioamnionitis (43%), alopecia (43%), neurodevelopmental and ophthalmologic abnormalities (36% each), tongue atrophy (29%), or a combination of these. Patients with CEVD may be prone to postnatal herpetic superinfections. Previously unreported findings included: erosive lichen planus, digital tip gangrene, and hydronephrosis.

The small patient sampling makes it difficult to define diagnostic criteria. As certain findings are associated with prematurity, it is unclear to what extent these features result from CEVD, premature birth, or another intrauterine pathology.

Although rare, CEVD should be considered in the differential diagnosis of neonatal vesicles/erosions in the context of a negative infectious workup. This review strengthens the spectrum of CEVD features, thus facilitating its recognition by clinicians.