Congenital erosive and vesicular dermatosis

Medical condition

Congenital erosive and vesicular dermatosis

Specialty| Dermatology

Congenital erosive and vesicular dermatosis is a cutaneous condition characterized by generalized erosions, vesicles, crusting and 'scalded skin-like' erythematous areas affecting up to 75% of the body surface area.[1]

## Signs and symptoms

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Congenital erosive and vesicular dermatosis exhibits erythema, vesicles, erosions, crusts, and fissures affecting more than 75% of the skin at birth. Within ten days to three months, the lesions heal on their own, leaving behind a pathognomonic reticulate scar.[2]

Clinical characteristics linked to congenital erosive and vesicular dermatosis include cicatricial baldness (39%), nail dysplasia or hypoplasia (39%), hyperthermia with/or hypohidrosis in scarred areas (39%), ophthalmological abnormalities (36%), and neurodevelopmental abnormalities (30%).[3]

## Causes

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There is no established pathophysiology for congenital erosive and vesicular dermatosis, and the illness seems to be sporadic. Intrauterine infections, amniotic adhesions, and a developmental abnormality with atypical repair in preterm skin are among the etiologic possibilities.[4]

## Diagnosis

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The congenital erosive and vesicular dermatosis histopathologic results vary according to the disease's stage.[4] Epidermal necrosis, subepidermal vesiculation, and an eroded epidermis with a primarily neutrophilic or mixed (containing eosinophils, histiocytes, lymphocytes, and neutrophils) dermal infiltration have all been reported in early inflammatory lesions.[3] Biopsy specimens from late lesions might either look normal in the dermis or exhibit scar development with a decrease in hair follicles and absent eccrine glands, according to histopathologic evaluation.[5]

## See also

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* Melanotic neuroectodermal tumor of infancy
* List of cutaneous conditions

## References

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1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
2. ^ Palmeiro, Ana Gusmão; Silva, Leandro; Lança, Ana; Amaro, Cristina (2022). "Congenital erosive and vesicular dermatosis: an atypical presentation of a rare dermatosis". BMJ Case Reports. 15 (3). BMJ: e247909. doi:10.1136/bcr-2021-247909. ISSN 1757-790X. PMC 8889243.
3. ^ a b Wong, Vincent; Fischer, Gayle (2017). "Congenital erosive and vesicular dermatosis with reticulated supple scarring". Australasian Journal of Dermatology. 58 (4). doi:10.1111/ajd.12579. ISSN 0004-8380.
4. ^ a b Tlougan, Brook E.; Paller, Amy S.; Schaffer, Julie V.; Podjasek, Joshua O.; Mandell, Jenny A.; Nguyen, Xuan H.; Spraker, Mary K.; Hansen, Ronald C. (2013). "Congenital erosive and vesicular dermatosis with reticulated supple scarring: Unifying clinical features". Journal of the American Academy of Dermatology. 69 (6). Elsevier BV: 909–915\. doi:10.1016/j.jaad.2013.08.015. ISSN 0190-9622.909-915&rft.date=2013&rft_id=info:doi/10.1016/j.jaad.2013.08.015&rft.issn=0190-9622&rft.aulast=Tlougan&rft.aufirst=Brook+E.&rft.au=Paller,+Amy+S.&rft.au=Schaffer,+Julie+V.&rft.au=Podjasek,+Joshua+O.&rft.au=Mandell,+Jenny+A.&rft.au=Nguyen,+Xuan+H.&rft.au=Spraker,+Mary+K.&rft.au=Hansen,+Ronald+C.&rfr_id=info:sid/en.wikipedia.org:Congenital+erosive+and+vesicular+dermatosis" class="Z3988">
5. ^ Plantin, P. (1990-04-01). "Congenital Erosive Dermatosis With Reticulated Supple Scarring: First Neonatal Report". Archives of Dermatology. 126 (4): 544. doi:10.1001/archderm.1990.01670280130035. ISSN 0003-987X.

## Further reading

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* Lee, Ji Hyun; Yoon, Sun Young; Lee, Jeong Deuk; Cho, Sang Hyun (2008). "A case of congenital erosive and vesicular dermatosis with limited involvement". Journal of the American Academy of Dermatology. 58 (5). Elsevier BV: S104 – S106. doi:10.1016/j.jaad.2007.03.038. ISSN 0190-9622.S104+-+S106&rft.date=2008&rft_id=info:doi/10.1016/j.jaad.2007.03.038&rft.issn=0190-9622&rft.aulast=Lee&rft.aufirst=Ji+Hyun&rft.au=Yoon,+Sun+Young&rft.au=Lee,+Jeong+Deuk&rft.au=Cho,+Sang+Hyun&rfr_id=info:sid/en.wikipedia.org:Congenital+erosive+and+vesicular+dermatosis" class="Z3988">
* Sadick, Neil S; Shea, Christopher R; Schlessel, Jerrold S (1995). "Congenital erosive and vesicular dermatosis with reticulated, supple scarring: A neutrophilic dermatosis". Journal of the American Academy of Dermatology. 32 (5). Elsevier BV: 873–877\. doi:10.1016/0190-9622(95)91551-6. ISSN 0190-9622.873-877&rft.date=1995&rft_id=info:doi/10.1016/0190-9622(95)91551-6&rft.issn=0190-9622&rft.aulast=Sadick&rft.aufirst=Neil+S&rft.au=Shea,+Christopher+R&rft.au=Schlessel,+Jerrold+S&rft_id=https://doi.org/10.1016%2F0190-9622%2895%2991551-6&rfr_id=info:sid/en.wikipedia.org:Congenital+erosive+and+vesicular+dermatosis" class="Z3988">

## External links

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* VisualDx

Classification|

* ICD-11: KC31
* ICD-10: Q82.8
* SNOMED CT: 773691007

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External resources|

* Orphanet: 231573
* Scholia: Q5160429