The subcellular localization of ERBIN in normal human skin is similar to that of Erb-B2 and varies with cell differentiation. Based on our findings and on the biological activities of Erb-B2, it is conceivable that disturbed expression or functioning of ERBIN and Erb-B2 is implicated in the development of the malignant phenotype of BCC.
Our patient presented features characteristic of PH. Although circulating anti-Dsg3 antibodies were present, the patient had only cutaneous involvement for a long period. Our findings indicate that the proposed Dsg compensation theory cannot always explain the clinical phenotype, changes in which may occur without apparent modification of the autoantibody profile and antibody specificity. Hence, additional factors, such as Fcgamma-dependent neutrophil activation, may critically affect the clinical presentation of pemphigus.
Background: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. Observation: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. Conclusion: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.
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.
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