Dermatosis erosive and vesicular congenital with reticular soft scarring

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

CEVD; Congenital erosive and vesicular dermatosis; Dermatosis, erosive and vesicular, congenital, with reticular soft scarring; ORPHA:231573

History
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Cohen et al. 1985

Definition
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Very rare (up to now < 50 patients have been described) congenital dermatosis of unexplained aetiology already present at birth with disseminated vesicles and erosions. The lesions heal within the first two years of life with formation of reticular scars.

In one case congenital hearing loss has been reported (Ciorba A et al. 2016), in another case congenital nasolacrimal atresia has been reported. Other systemic involvement has not been reported so far.

Therapy
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Symptomatic

Literature
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  1. Ciorba A et al (2016) Sensorineural Hearing Loss in Congenital Erosive and Vesicular Dermatosis. J Int Adv Otol 12:208-209.
  2. Kong BY et al (2018) Congenital erosive and vesicular dermatosis with reticulated supple scarring. Arch Dis Child Fetal Neonatal Ed 103: F78.
  3. Srinivas SM et al (2018) Congenital erosive and vesicular dermatosis healing with reticulated supple scarring: Report of four cases. Indian J Dermatol Venereol Leprol 84:73-75.
  4. Wong V et al (2017) Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. Australas J Dermatol 58:e236-e239.
  5. Yam JC et al (2014) Bilateral canalicular and nasolacrimal duct obstruction in congenital erosive and vesicular dermatosis: a case report and review of the literature. J AAPOS 18:88-90.

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Last updated on: 29.10.2020