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Congenital erosive and vesicular dermatosis

From Wikipedia, the free encyclopedia
Congenital erosive and vesicular dermatosis
SpecialtyDermatology

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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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.
  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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