Acquired Melanocytic Naevus in Childhood Vulval Pemphigoid

Abstract

Background: Eruptive epidermolysis bullosa (EB) naevi comprise a subset of melanocytic naevi with atypical features that characteristically occur in areas of former blistering in patients suffering from hereditary EB. Observation: The case is reported of a girl who presented with pruritus, blistering and erosions of the vulval region. Clinical and immunopathological features were consistent with the diagnosis of childhood vulval pemphigoid. In the course of the disease, she developed an atypical melanocytic naevus on the left labium at a site of former blistering. Although its clinical and dermoscopic features resembled malignant melanoma, the lesion completely regressed clinically during the 24-month follow-up. Conclusion: This is the first report describing the development of a melanocytic naevus at sites of blistering in an auto-immune subepidermal blistering disease in childhood. Our observation extends the spectrum of disorders, in addition to the group of congenital EB, in which ‘eruptive’ atypical melanocytic naevi may occur. Knowledge of this complication is important for appropriate management and follow-up and to avoid radical surgery.

Pigmented lesions in children: when to worry

“…Sun exposure is the primary environmental influence (Table 1) [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27], while hereditary components include pigmentary phenotype as well as a particular predisposition to 'moliness'.…”

Epidermolysis Bullosa Nevus

“…As in our patient, the lesion resembled melanoma clinically and dermoscopically. 17 Although the association between squamous cell carcinoma and chronic scarring inflammatory dermatosis is well known, it is unclear whether such an association exists for melanoma in general. Data suggest that a higher incidence of melanoma may exist in patients with EB, [18][19][20] especially recessive dystrophic EB, but there are no detailed, well-documented cases in the literature, in contrast to the well-documented and now well-known higher incidence of squamous cell carcinoma in patients with EB.…”

Nevo da epidermólise bolhosa: caso clínico e revisão da literatura

“…3,[9][10][11][12] There is only one report describing a child with bullous pemphigoid located on the a vulva, where recurrent blisters developed, an atypical melanocytic nevus with clinical and dermoscopic characteristics of melanoma. 13 This was the first case where a melanocytic nevus developed on the site of bullous lesions, in bullous autoimmune and subepidermal disease in childhood.…”