PA27 Expanding the phenotype of congenital erosive and vesicular dermatosis

Abstract Congenital erosive and vesicular dermatosis (CEVD) is a rare condition that typically presents at birth with erosions and vesicles (Cohen B, Esterly N, Nelson P. Congenital erosive and vesicular dermatosis healing with reticulated and supple scarring. Arch Dermatol 1985; 121: 361–7). Lesions heal with distinctive reticulate and supple scarring and can involve > 75% of the skin surface (Tlougan BE, Paller AM, Schaffer JV et al. Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features. J Am Acad Dermatol 2013; 69: 909–15). The pathogenesis of CEVD is unknown, although hypotheses include intrauterine infection, amniotic adhesions, or a developmental defect in skin healing. Histopathological findings early in the disease process can include epidermal necrosis, subepidermal vesiculation and an eroded epidermis. Older lesions demonstrate scar formation, decreased hair follicles, and absent eccrine glands. Historically, CEVD has been described as preferentially affecting the trunk and limbs, with sparing of the face and volar surfaces. Patients with CEVD have been reported to have higher prevalence of premature birth, neurodevelopmental abnormalities, nail abnormalities, tongue atrophy or sores, alopecia, and cardiac defects. Here, we highlight two patients with presumed CEVD with atypical features. Patient A presented at 13 months with a history of linear erosions and vesiculation over their scalp, forehead and posterior left flank. The lesions were present at birth and healed with reticulate scarring with no further flares. Patient B presented at 11 months with a history of stellate scarring over the lower abdomen, with noncontiguous involvement of the right and left axillae, scalp, and right forehead. The lesions were also present at birth and were initially more erythematous and developed milia-like cysts. The cysts resolved, leaving reticulate and supple scarring. Of note, patient B had no erosions or vesicles. At the time of assessment, both children were otherwise healthy and developing normally. These cases add to evidence that CEVD is neither facial nor scalp sparing, as commonly described, and can be more localized than previously thought, but affect noncontiguous sites. Case B also demonstrates that, despite the name, CEVD can present without erosions, and instead with milia-like cysts. The cases also demonstrate that CEVD can occur in the absence of other congenital abnormalities; outcomes for children with the condition are then more favourable. Both families were provided with reassurance that although the scarring would not disappear completely, and can be of cosmetic concern, CEVD is a nonprogressive condition and thus no specific treatment is indicated.

Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.