Lichenoid erythrodermic bullous pemphigoid of the African patient.
BACKGROUND
Lichen planus (LP) pemphigoides is defined by the concurrence of bullous pemphigoid (BP) and LP lesions. However, other autoimmune bullous skin diseases such as paraneoplastic pemphigus or bullous lupus erythematosus may also be associated with lichenoid eruptions.


OBJECTIVE
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. The ultrastructural localization of autoantibody deposits was studied by direct and indirect immunoelectron microscopy. The antigenic specificities recognized by the patients' sera were analyzed by immunoblotting 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 detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype.


CONCLUSIONS
The clinical and histologic features of these 3 patients were distinct from classic LP pemphigoides. Their common geographic origin and the presence of the HLA-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.
Joly
P
P
Research Group on Immunopathology, IFR 23, Hôpital Charles Nicolle, Rouen, France.
Tanasescu
S
S
Wolkenstein
P
P
Bocquet
H
H
Gilbert
D
D
Thomine
E
E
Wechsler
J
J
Roujeau
J C
JC
Revuz
J
J
Tron
F
F
Lauret
P
P
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D001324:Autoantigens / Q000032:analysis; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D003873:Dermatitis, Exfoliative / Q000276:immunology / Q000473:pathology*; D003896:Desmosomes / Q000276:immunology / Q000648:ultrastructure; D000071821:Dystonin; D004817:Epidermis / Q000276:immunology / Q000473:pathology; D000939:Epitopes; D019085:Fluorescent Antibody Technique, Direct; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis; D017512:Lichenoid Eruptions / Q000276:immunology / Q000473:pathology*; D008297:Male; D008435:Mauritania; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D009059:Mouth Diseases / Q000276:immunology / Q000473:pathology; D009061:Mouth Mucosa / Q000276:immunology / Q000473:pathology; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000276:immunology / Q000473:pathology*; D012675:Senegal; D012867:Skin / Q000276:immunology / Q000473:pathology; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D000939:Epitopes; D006684:HLA-DR Antigens; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human