LICHENOID ERYTHRODERMIC BULLOUS PEMPHIGOID OF THE AFRICAN PATIENT

Background: Lichen planus (LP) pemphigoides is defined by the concurre nce of bullous pemphigoid (BP) and LP lesions. However, other autoimmu ne bullous skin diseases such as paraneoplastic pemphigus or bullous l upus erythematosus may also be associated with lichenoid eruptions. Ob jective: Our purpose was to describe 3 African patients with a similar form of lichenoid erythrodermic BP. Methods: Medical records of the 3 patients were reviewed for clinical, histologic, and biologic data. T he ultrastructural localization of autoantibody deposits was studied b y direct and indirect immunoelectron microscopy. The antigenic specifi cities recognized by the patients' sera were analyzed by immunoblottin g on human epidermal extracts. Results: Three black African men had a severe lichenoid erythroderma associated with BP lesions involving the skin and mucosa. Histologic and direct immunofluorescence examination of skin and mucosal lesions were consistent with the diagnosis of BP associated with a lichenoid dermatitis. Immunoblot analysis of sera de tected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microsc opy showed IgG deposits localized in the lamina lucida and the hemides mosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. Conclusion: The clinical and h istologic features of these 3 patients were distinct from classic LP p emphigoides. Their common geographic origin and the presence of the HL A-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.