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- Presentation
Rare Neonatal Dermatologic Disorders: Congenital Erosive Vesicular Dermatosis and Hay-Wells Syndrome
Description
The lecture reviewed two rare neonatal dermatologic disorders using illustrative cases. The first involved premature twins with congenital erosive and vesicular dermatosis with reticulate scarring, presenting at birth with erythema, erosions, and later reticulated scars, gluteal involvement, scalp scarring, and areas of hypopigmentation; one twin had skin herniations. Workup showed mostly normal labs and histopathology compatible with the diagnosis, including subepidermal vesicles and dermal sclerosis. The condition was discussed as an uncommon, poorly understood disorder often seen in preterm infants, possibly linked to congenital infections such as herpes simplex, and generally managed supportively with a good long-term prognosis. The second case was a preterm infant with cleft palate, ankyloblepharon, erythroderma, erosions, and ectodermal findings. Genetic testing identified a pathogenic TP63 variant, confirming Hay-Wells syndrome, a rare genodermatosis that affects epithelial and ectodermal development. The talk emphasized the need to differentiate these entities from epidermolysis bullosa, incontinentia pigmenti, aplasia cutis, herpes infection, and staphylococcal scalded skin syndrome, and highlighted the importance of clinicopathologic correlation, genetic testing, and multidisciplinary care.
View moreConclusions
- Congenital erosive and vesicular dermatosis is a rare neonatal condition, seen mainly in preterm infants, whose cause remains unclear but may sometimes be associated with congenital herpes infection.
- CEVD typically begins at birth with widespread erythema, erosions, crusting, and sometimes vesicles, then improves spontaneously over weeks to months and leaves reticulated scarring.
- The diagnosis of CEVD depends on combining the clinical pattern with histopathology, which shows early epidermal necrosis and subepidermal blistering and later scar tissue with reduced skin appendages.
- The long-term prognosis of CEVD is generally favorable, and supportive care is usually sufficient.
- Hay-Wells syndrome is another rare neonatal genodermatosis caused by TP63 mutations and characterized by ankyloblepharon, cleft palate, skin erosions, and ectodermal defects.
- Definitive diagnosis of Hay-Wells syndrome requires both recognition of the clinical hallmarks and genetic confirmation of a pathogenic TP63 variant.
- Management of Hay-Wells syndrome requires multidisciplinary care, especially dermatology, surgery, dentistry, and genetic counseling.
- Both CEVD and Hay-Wells syndrome are challenging neonatal diagnostic entities, and complementary studies such as histopathology and genetic testing are essential for reaching the correct diagnosis.
- Tlougan, Brook E., et al. “Congenital erosive and vesicular dermatosis with reticulated supple scarring: Unifying clinical features.” Journal of the American Academy of Dermatology, Vol. 69, Issue 6, pp. 909–915.#10.1016/j.jaad.2013.08.015
- Leila Nemazee, Laura Cuddy, Timothy H. Clayton. “Congenital erosive and vesicular dermatosis with reticulated scarring in twins, associated with oesophageal atresia.” Clinical and Experimental Dermatology.#10.1111/ced.15033
- Rositto et al. (2011). Article on congenital erosive and vesicular dermatosis.#10.32388/ewzzfl
- Palmeiro et al. (2022). BMJ Case Report on congenital erosive and vesicular dermatosis.#10.32388/ewzzfl
- Moxham, Felton, and Lam (2025). Pediatr Dermatol paper on congenital erosive and scarring eruption.
- Nemazee et al. (2022). Clinical and Experimental Dermatology report on twins with reticulated scarring and esophageal atresia.
- McGrath J.A., et al. Hay-Wells syndrome and p63.
- Serra G., et al. TP63 mutation in a newborn with AEC syndrome.
- Payne A.S., et al. Two novel TP63 mutations associated with AEC syndrome and skin fragility.