Congenital erosive and vesicular dermatosis healing with reticulated supple scarring: Report of four cases

Widespread skin erosions in a newborn

“…5 Though our patient showed some atypical features of CEVD, including term birth and involvement of the soles, there are 10 reported cases in term infants and two previous cases involving the palms or soles. 1,5 Consistent with our patient's biopsy from the leg, histopathologic features of early CEVD may include necrosis or erosion of the epidermis, subepidermal vesiculation, and a neutrophilic or mixed infiltrate within the dermis. 5 While our patient's biopsy from the arm showed epidermal features more typical of epidermolytic ichthyosis (EI) than CEVD, including hyperkeratosis, hypergranulosis, and a focal area suggestive of epidermolytic hyperkeratosis, 7 our patient has not demonstrated clinical findings of hyperkeratosis or epidermolytic ichthyosis on follow-up.…”

“…Additional clinical features of CEVD may include nail hypoplasia or dysplasia, hyperthermia, hypohidrosis, scarring alopecia, ocular abnormalities, or neurodevelopmental abnormalities . Though our patient showed some atypical features of CEVD, including term birth and involvement of the soles, there are 10 reported cases in term infants and two previous cases involving the palms or soles …”

“…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 . However, two pairs of non‐twin siblings, each from consanguineous parents, were recently reported to have CEVD . This raises suspicion of a genetic predisposition or recurrent intrauterine pathology leading to CEVD.…”

“…Congenital erosive and vesicular dermatosis is a rare congenital dermatosis of unknown etiology, with only 36 cases reported thus far. 1 CEVD most commonly affects premature infants and presents at birth with superficial erosions and vesicles, typically involving a large body surface area, with relative sparing of volar surfaces. [1][2][3][4][5] Lesions heal over several months with characteristic supple and reticulated scarring.…”

“…1 CEVD most commonly affects premature infants and presents at birth with superficial erosions and vesicles, typically involving a large body surface area, with relative sparing of volar surfaces. [1][2][3][4][5] Lesions heal over several months with characteristic supple and reticulated scarring. 5,6 Additional clinical features of CEVD may include nail hypoplasia or dysplasia, hyperthermia, hypohidrosis, scarring alopecia, ocular abnormalities, or neurodevelopmental abnormalities.…”