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Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014
Online ISSN 1827-1820
Fabbri P., Fuligni A., Amato L. E., Caproni M.
Università degli Studi - Firenze, Istituto di Clinica Dermosifilopatica, Clinica Dermatologica II
Cicatricial pemphigoid (CP) is a rare not hereditary chronic blistering disease of DEJ that primarily affects the mucous membranes (most frequently the oral and conjunctival mucous membranes). Skin lesions are less frequent and characterized by the presence of tight blisters that quickly break leading to a superficial abrasion in which the evolution results in an atrophic-scar formation. Classically there are 3 variants: CP with exclusive mucous involvement, CP with mucous and cutaneous involvement, CP with exclusive cutaneous involvement. The more significant histopathological finding of CP is represented by a non acantholytic subepidermal blister in association with an inflammatory infiltrate in the submucosa or in the superficial dermis. Characteristic immunopathological findings are the presence of circulating IgG antibodies (30% of the cases) directed against antigens found within the lamina lucida and linear IgG and C3 deposition along the DEJ or between the mucosa and submucosa (90% of the cases). The most sensitive technique for the demonstration of these circulating antibodies is IIF performed on normal human split-skin that, in the majority of the cases, reveals an epidermal or dermal-epidermal pattern. Immunoelectron microscopy shows deposition of IgG either in the deepest part of the lamina lucida, at the lamina densa, or in the region right underneath the hemidesmosomes. The bullae are probably due to either the activity of the specific IgG antibodies or to auto-reactive T lymphocytes CD4+. The antigens of CP are a 230 Kd intrahemidesmosomial glycoprotein and a 180 Kd transhemidesmosomial glycoprotein; furthermore in 5% of the patients antibodies can recognize a 165 Kd antigenic structure, identified with the epilegrin in the α3 subunit. Other more recent researches have instead attempted to define the cytokines released by the T lymphocytes CD4+ and responsable of the fibrosis and the following atrophic-scarring evolution that is often destructive and debilitating.