Congenital Erosive and Vesicular Dermatosis Healing with Reticulated Scarring

Abstract

International audienceno abstrac

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

“…There has been only one other case of CEVD described in identical twins, 1 with the majority of cases described in children who were not twins. There are two previous cases of CEVD with associated bronchodysplasia, 5,6 therefore it is more than probable that the first twin in this case also had CEVD and thus either an infective or genetic causation is possible. The twins were heterozygous, but given the rarity of CEVD, their concordance does seem significant.…”

Congenital erosive and vesicular dermatosis healing with reticulated and supple scarring: Two cases secondary to fungal infection

“…[2][3][4]6,[9][10][11][12][13] Adhesive membranes have been observed in 12% of patients with CEVD, and remnants that are removed leave behind erosions. 2,6,14 However, this does not explain the unique pattern of healing. 5 Instead, it is more likely that CEVD occurs due to an intrauterine, infectious pathology with premature, nonfunctional skin playing a role in the characteristic RSS pattern.…”

“…Exclusion of fungal infection is of particular importance. Of the 37 cases of CEVD currently reported in the literature, only three were evaluated for fungal infection as the potential cause 10,14 . Ten of the physical exams described had thick crust and scale consistent with CCC, and two patients went on to develop candidemia 1,3,4,6–9,12,15,16 .…”

Congenital erosive and vesicular dermatosis with reticulated supple scarring

“…Intrauterine bacterial or viral infections had previously been proposed as a possible aetiology . In a recent case of CEVD Staphylococcus haemolyticus (thought to be nosocomial) was detected . Another patient had concurrent HSV 1 infection at birth, confirmed by polymerase chain reaction and a positive immunoglobulin (Ig) G titre .…”

“…The lesions subsequently heal within 10 days to 3 months, leaving supple scarring of a symmetrical reticulated pattern consisting of elevated bands of normal to minimally decreased pigmentation and intervening depressed areas of normal to minimally increased pigmentation . Clinical features associated with CEVD include nail hypoplasia or dysplasia (39%), hyperthermia and/or hypohidrosis in scarred areas (39%), cicatricial alopecia (39%), ophthalmological abnormalities (36%) and neurodevelopmental abnormalities (30%) …”