Congenital erosive and vesicular dermatosis with reticulated supple scarring: A report of two new cases

Congenital erosive and vesicular dermatosis with reticulated supple scarring (CEVD) is a rare cutaneous disorder of unknown aetiology. Only 32 cases are documented in the literature. It is strongly associated with preterm birth, and classically presents as superficial erosions and vesicles at birth that often involve more than 75% of the body surface area, with relative sparing of the face and volar surfaces.1 The lesions subsequently heal within ten days to three 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.
We report two new cases of CEVD with atypical presentations. The first patient presented at birth with widespread petechial patches, which were neither eroded nor blistered, in a reticulated distribution most extensive on the trunk but also affecting her right cheek and forehead. To the best of our knowledge, the absence of erosions and vesicles on initial presentation has not been previously reported. The patient subsequently experienced sporadic blistering that continued until the age of 15 months, after which she was lost to follow-up. The recurrence of vesicles beyond the neonatal period is a feature that occurred in less than one-quarter of previously documented cases. Our second patient is the second documented case to develop CEVD after birth, in the form of erythematous plaques with some vesicles.2 He presented to our dermatology outpatient clinic at eight months of age with two symmetrical, well-demarcated hypopigmented scars along his back.
1. Cohen BA, Esterly NB, Nelson PF. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. Arch Dermatol 1985; 121: 361-7.
2. Vun YY, Malik MM, Murphy GM, et al. Congenital erosive and vesicular dermatosis. Clin Exp Dermatol 2005; 30: 146-8.

Dr. Vincent Wong

Dept of Geriatrics, Westmead Hospital

I am a JMO at Westmead Hospital, with a keen interest in skin disease and research.