Congenital Erosive and Vesicular Dermatosis Healing With Reticulated Supple Scarring

Widespread skin erosions in a newborn

“…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, our patient has not demonstrated clinical findings of hyperkeratosis or epidermolytic ichthyosis on follow‐up. Though not performed for our patient, biopsy of late‐stage CEVD shows scar formation and reduction of hair follicles and eccrine glands . Electron microscopy has not demonstrated consistent findings, and the results of direct immunofluorescence are nonspecific …”

“…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 . Lesions heal over several months with characteristic supple and reticulated scarring . Additional clinical features of CEVD may include nail hypoplasia or dysplasia, hyperthermia, hypohidrosis, scarring alopecia, ocular abnormalities, or neurodevelopmental abnormalities .…”

Congenital erosive and vesicular dermatosis

“…Biopsies of older or healed lesions have shown variable changes in the follicular structures 1,2 . There was an absence of eccrine glands in five of the biopsies 2,3 , 5 . This corresponds to the hypohidrosis reported in some patients 3 .…”

“…Cohen et al . reported one affected identical twin, and no family member has been reported to be affected so far 3 …”

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring with extensive facial involvement

“…Editor Congenital erosions and vesicles dermatosis healing with reticulate scarring is a rare disease first reported by Cohen et al. 1 To date only 16 cases of this disease have been reported 2–5 . This disease most severely affects the trunk and extremities, with relative sparing of the face and volar surfaces.…”