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.

Keywords:
Childhood vulval pemphigoid, Pemphigoid, Naevus, atypical, Dermoscopy, Epidermolysis bullosa
1.
Laffitte E, Borradori L: Bullous pemphigoid and related disorders; in Hertl M (ed): Autoimmune Diseases of the Skin. Vienna, Springer, 2001, pp 63–83.
2.
Nemeth AJ, Klein AD, Gould EW, et al: Childhood bullous pemphigoid: clinical and immunologic features, treatment, and prognosis. Arch Dermatol 1991;127:378–386.
3.
Fisler RE, Saeb M, Liang MG, Howard RM, McKee PH: Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. Am J Dermatopathol 2003;25:183–189.
4.
Farrell AM, Kirtschig G, Dalziel KL, et al: Childhood vulval pemphigoid: a clinical and immunopathological study of five patients. Br J Dermatol 1999;140:308–312.
5.
Saad RM, Domolge-Hultsch N, Yancey KB, Benson PM, James WD: Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid. Arch Dermatol 1992;128:807–810.
6.
Johr RH: Dermoscopy: alternative melanocytic algorithms – The ABCD rule of dermatoscopy, Menzies scoring method, and 7-point checklist. Clin Dermatol 2002;20:240–247.
7.
Hashimoto I, Schnyder UW, Anton-Lamprecht I: Epidermolysis bullosa hereditaria with junctional blistering in an adult. Dermatologica 1976;152:72–86.
8.
Bauer JW, Schaeppi H, Kaserer C, Hantich B, Hintner H: Large melanocytic nevi in hereditary epidermolysis bullosa. J Am Acad Dermatol 2001;44:577–584.
9.
Stavrianeas NG, Katoulis AC, Moussatou V, et al: Eruptive large melanocytic nevus in a patient with hereditary epidermolysis bullosa simplex. Dermatology 2003;207:402–404.
10.
Bichel J, Metze D, Bruckner-Tudermann L, Stauder S: Large melanocytic nevi in generalized atrophic benign epidermolysis bullosa (epidermolysis bullosa nevi). Hautarzt 2001;52:812–816.
11.
Hoss DM, McNutt NS, Carter DM, et al: Atypical melanocytic lesions in epidermolysis bullosa. J Cutan Pathol 1994;21:164–169.
12.
Christophoridis S, Budinger L, Borradori L, et al: IgG, IgA and IgE autoantibodies against the ectodomain of BP180 in patients with bullous and cicatricial pemphigoid and linear IgA bullous dermatosis. Br J Dermatol 2000;143:349–355.
13.
Virgili A, Zampino MR, Corazza M: Primary vulvar melanoma with satellite metastasis: dermoscopic findings. Dermatology 2004;208:145–148.
14.
Grubauer G, Hintner H, Klein G, Fritsch P: Acquired, surface giant nevus cell nevi in generalized, atrophic, benign epidermolysis bullosa. Hautarzt 1989;40:523–526.
15.
Soltani K, Pepper MC, Simjee S, Apatoff B: Large acquired nevocytic nevi induced by the Koebner phenomenon. J Cutan Pathol 1984;11:296–299.
16.
Lanschuetzer CM, Emberger M, Laimer M, Diem A, Bauer JW, Soyer HP, Hintner H: Epidermolysis bullosa naevi reveal a distinctive dermoscopic pattern. Br J Dermatol 2005;153:97–102.
17.
Kopf AW, Grupper C, Baer L, Mitchell JC: Eruptive nevocytic nevi after severe bullous disease. Arch Dermatol 1977;113:1080–1084.
18.
Goerz G, Tsambaos D: Eruptive nevocytic nevi after Lyell’s syndrome. Arch Dermatol 1978;114:1400–1401.
19.
Shoji T, Cockerell CJ, Koff AB, Bhawan J: Eruptive melanocytic nevi after Stevens-Johnson syndrome. J Am Acad Dermatol 1997;37:337–339.
20.
Fine JD, Eady RA, Bauer EA, Briggaman RA, Bruckner-Tuderman L, Christiano A, Heagerty A, Hintner H, Jonkman MF, McGrath J, McGuire J, Moshell A, Shimizu H, Tadini G, Uitto J: Revised classification system for inherited epidermolysis bullosa: report of the Second International Consensus Meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol 2000;42:1051–1066.
21.
Mattei S, Colombo MP, Melani C, et al: Expression of cytokine/growth factors and their receptors in human melanoma and melanocytes. Int J Cancer 1994;56:853–857.
22.
Giomi B, Caproni M, Calzolari A, Bianchi B, Fabbri P: Th1, Th2 and Th3 cytokines in the pathogenesis of bullous pemphigoid. J Dermatol Sci 2002;30:116–128.
23.
Lanschuetzer CM, Emberger M, Hametner R, et al: Pathogenic mechanisms in epidermolysis bullosa naevi. Acta Derm Venereol 2003;83:332–337.
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2006
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