Congenital erosive and vesicular dermatosis with reticulated supple scarring

Widespread skin erosions in a newborn

“…5,8 Proposed etiologies of CEVD include in utero infection, amniotic adhesions, and developmental defects. 9 The role of HSV remains unclear, as there are two reported cases of CEVD with congenital HSV infection detected at birth. 10 The skin erosions of CEVD may predispose to HSV inoculation, as several cases of postnatal HSV have been reported in neonates with CEVD.…”

“…Congenital erosive and vesicular dermatosis has been considered a sporadic condition due to the lack of family history, including two cases of monozygotic twins reported with only one twin in each set presenting with CEVD. 6,9 However, two pairs of non-twin siblings, each from consanguineous parents, were recently reported to have CEVD. 1 This raises suspicion of a genetic predisposition or recurrent intrauterine pathology leading to CEVD.…”

“…Proposed etiologies of CEVD include in utero infection, amniotic adhesions, and developmental defects . The role of HSV remains unclear, as there are two reported cases of CEVD with congenital HSV infection detected at birth .…”

“…This raises suspicion of a genetic predisposition or recurrent intrauterine pathology leading to CEVD. Another genomic theory is that CEVD may be the result of a post‐zygotic mutation, which could be supported by the cases of monozygotic twins with only one twin effected . As CEVD is a rare condition, the description of more cases may help to further elucidate the pathogenesis of this disease.…”

Congenital erosive and vesicular dermatosis with reticulated scarring in twins, associated with oesophageal atresia

“…Clinically, this is described as bands of elevated and depressed skin with varying areas of hyperpigmentation and hypopigmentation. 3 Currently, the condition appears sporadic and the pathogenesis remains unknown. Proposed aetiologies include intrauterine infections, amniotic adhesions, vascular aetiology and developmental defects.…”