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. OBSERVATION: 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.

[Localized cicatricial bullous pemphigoid of the Brunsting-Perry type]. / Lokalisiertes vernarbendes bullöses Pemphigoid vom Typ Brunsting-Perry.

Localized cicatricial pemphigoid of the Brunsting-Perry type is a very rare bullous condition, which has so far been reported in 51 cases. It is characterized by scarring blisters confined to the head, scalp and neck. Diagnosis can be difficult because of the discrete skin lesions, often repeatedly false-negative direct immunofluorescence, and the absence of circulating antibodies. We report on a 87-year-old male patient with the typical clinical feature of a cicatricial pemphigoid of the Brunsting-Perry type and give a review of the 51 cases published in the world literature.

Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid.

A 56-year-old woman with the typical clinical feature of cicatricial bullous pemphigoid of the Brunsting-Perry type was studied. Histologic examination of a lesion skin biopsy specimen demonstrated a subepidermal blister. Direct immunofluorescence microscopy revealed linear deposits of IgG, IgM, and C3 located on both the roof and the floor of the blister. Immunofluorescence antigen mapping using cryostat sections of a spontaneous blister and antisera against defined basement membrane components localized the bullous pemphigoid antigen and type IV collagen in the roof of the blister. This dermal type of blister formation was confirmed by electron microscopy, which showed the cleavage level below the lamina densa. In direct immunoelectron microscopy, granular deposits of C3 and IgG were found attached to and just beneath the lamina densa in a pattern identical to the distribution of anchoring fibrils. These findings are diagnostic of acquired epidermolysis bullosa, a blistering disease that has much more clinical heterogeneity than previously suggested.

Clinical association of autoantibodies to fibrillarin with diffuse scleroderma and disseminated telangiectasia.

Circulating autoantibodies against a variety of nuclear and nucleolar antigens are characteristic serologic findings in systemic scleroderma. Some of these antibodies correlate with clinical subsets of the disease. We describe three patients with systemic scleroderma and high autoantibody titers against U3 ribonucleoprotein-associated fibrillarin, a recently identified 34 kD nucleolar protein. These patients showed a progressive course with multiple organ and diffuse skin involvement with disseminated telangiectasia.

[The basement membrane and its significance in congenital and acquired diseases of the skin]. / Die Basalmembran und ihre Bedeutung bei angeborenen und erworbenen Erkrankungen der Haut.

Basal membranes are an ubiquitous component of all human organs and fulfil a large variety of functions. They separate epithelial from mesenchymal tissue and control the passage of substances, of inflammatory as well as tumor cells. They form the extracellular cytoskeleton, regulate growth processes, and play an important part in wound healing. Ultrastructurally, the lamina densa can be distinguished from the lamina lucida. Anchoring fibrils connect the basal membrane of the dermoepidermal junction with the underlying dermis, while anchoring plates connect it with the epidermal cells. During the last few years, various components of the basal membrane have been biochemically analyzed. Different proteins were characterized, and their corresponding morphological structure could be identified. The growing knowledge regarding the structure and composition of the dermo-epidermal junction has led to a better understanding of many diseases involving this structure, in particular inborn and acquired bullous diseases, but also that of the role of the basal membrane in metastasis and tumor invasions. The characterization of antigens in bullous diseases with circulating antibodies has led to exact diagnostic criteria concerning the often overlapping disorders and allows, e.g., the differentiation between bullous pemphigoid and epidermolysis bullosa acquisita.