Localized cicatricial pemphigoid of the Brunsting-Perry type with transition into disseminated cicatricial pemphigoid. Report of a case proved by preembedding immunogold electron microscopy.
BACKGROUND
In 1979, Provost described two patients with the clinical features of disseminated cicatricial pemphigoid for the first time. Until now, only four additional cases of disseminated cicatricial pemphigoid have been described. Existence of diagnosis of disseminated cicatricial pemphigoid has been discussed controversially because in four cases investigated by electron microscopy the blister formation was found below the lamina densa, which is indicative of an epidermolysis bullosa acquisita.


METHODS
A 78-year-old woman is presented with a generalized eruption of blisters leaving behind scars that developed after a 7-year-long history of mild circumscribed recurrent blisters and scarring eruptions that had been diagnosed previously as Brunsting-Perry type of cicatricial pemphigoid. Immunofluorescence antigen mapping disclosed the blister formation above the lamina densa. Electron and immunoelectron microscopy using a preembedding immunogold technique revealed blister formation and antibody binding within the lamina lucida, predominantly below the subbasal dense plate.


CONCLUSIONS
The clinical features of disseminated blistering followed by scarring, the immunofluorescence antigen mapping, and the electron and immunoelectron microscopic findings in our case for the first time clearly prove the existence of a disseminated cicatricial pemphigoid.
Kurzhals
G
G
Department of Dermatology and Allergology, City Hospital of Munich-Schwabing, Germany.
Stolz
W
W
Maciejewski
W
W
Karpati
S
S
Meurer
M
M
Breit
R
R
D000368:Aged; D001284:Atrophy; D001485:Basement Membrane / Q000473:pathology; D001768:Blister / Q000473:pathology; D002921:Cicatrix / Q000473:pathology; D003176:Complement C3 / Q000032:analysis; D018450:Disease Progression; D005148:Facial Dermatoses / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007150:Immunohistochemistry; D008854:Microscopy, Electron; D016253:Microscopy, Immunoelectron; D010390:Pemphigoid, Benign Mucous Membrane / Q000473:pathology* / Q000503:physiopathology; D012536:Scalp Dermatoses / Q000473:pathology* / Q000503:physiopathology; D016610:Tissue Embedding
D003176:Complement C3; D007074:Immunoglobulin G