| 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
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
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
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
References
- ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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
- 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.
- 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.