Localized Cicatricial Pemphigoid of the Brunsting-Perry Type With Transition Into Disseminated Cicatricial Pemphigoid

Kurzhals, Günter; Stolz, Wilhelm; Maciejewski, Wojciech; Kárpáti, Sarolta; Meurer, Michael; Breit, Reinhard

doi:10.1001/archderm.1995.01690170082012

Cited by 29 publications

(12 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recent investigations have demonstrated that the causative antigen is the BP180 molecule, 2–4 which is the pathological antigen in cases of bullous pemphigoid (BP) and gestational pemphigoid, and seems to be related to some cases of mucous membrane pemphigoid and some linear IgA disease (LAD). The finding that the causative antigen is BP180 concurs with the previous finding obtained from immunoelectronmicroscopy that immune deposits in this type of pemphigoid are distributed in the lamina lucida, especially beneath the hemidesmosomes 5,6 . However, a few cases have been also reported to show clinical features very similar to those of Brunsting–Perry type pemphigoid, whereas the cleavage and immune deposition were at the sub‐lamina densa area, suggesting that those should be diagnosed as epidermolysis bullosa acquisita (EBA) 7,8 .…”

Section: Introductionsupporting

confidence: 90%

“…However, it is also possible to think that Brunsting–Perry type pemphigoid is heterogeneous. Considering the findings of previous reports of Bursting–Perry type pemphigoid in which the cleavage level and location of immune deposits were discussed, it should be concluded that on one side of this disease, there are cases in which pathogenic mechanisms involve the lamina lucida, 2–6 like BP, and some cases of mucous membrane pemphigoid, and on the other side are cases in which these mechanisms occur beneath or along the lamina densa, 7–9 like EBA. Our case represents an intermediate form of Brunsting–Perry type pemphigoid between BP and EBA.…”

Section: Discussionmentioning

confidence: 93%

See 1 more Smart Citation

Heterogeneity of Brunsting-Perry type pemphigoid: A case showing blister formation at the lamina lucida, immune deposition beneath the lamina densa and autoantibodies against the 290-kD polypeptide along the lamina densa

Minato

Ishii

Fukuda

et al. 2011

The Journal of Dermatology

View full text Add to dashboard Cite

An otherwise healthy 31-year-old man presented with multiple, vesicular, subepidermal blistering on the head, face, chest and oral cavity, leaving shallow scar formation, typical of Brunsting-Perry type pemphigoid. Direct immunofluorescence showed linear deposition of immunoglobulin (Ig)G and C3 along the basement membrane zone (BMZ), and indirect showed anti-BMZ autoantibodies (IgG, >40×) reacting with the dermal side under the salt-split study. Immunofluorescence staining for type IV collagen and laminins, as well as routine electron microscopy, demonstrated that the cleavage level of the blister was intra-lamina lucida. The immunoperoxidase method applied to lesional skin demonstrated IgG deposits along the lamina densa. The post-embedding immunogold method demonstrated that the autoantibodies against BMZ reacted with the lamina densa and the dermis just beneath it. Immunoblot studies demonstrated that the autoantibodies reacted with the 290-kD polypeptide (suggesting type VII collagen) when dermal extract was used as the substrate. The patient was treated with combination therapy consisting of 30 mg prednisolone, 900 mg nicotinamide and 750 mg tetracycline, and the number of newly forming blisters decreased. We concluded that Brunsting-Perry type pemphigoid, a rare autoimmune blistering disease, includes cases showing characteristics of epidermolysis bullosa acquisita as well as bullous pemphigoid. This case showed discrepancy between the blistering level (intra-lamina lucida) and location of antigen (lamina densa and sub-lamina densa areas).

show abstract

Section: Introductionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 93%

Heterogeneity of Brunsting-Perry type pemphigoid: A case showing blister formation at the lamina lucida, immune deposition beneath the lamina densa and autoantibodies against the 290-kD polypeptide along the lamina densa

Minato

Ishii

Fukuda

et al. 2011

The Journal of Dermatology

View full text Add to dashboard Cite

show abstract

“…Brunsting–Perry type BP appears to be a heterogeneous disease and may present a clinical feature of localized epidermolysis bullosa acquisita (EBA) . However, autoantigens or antigenic epitopes in most cases of Brunsting–Perry type BP remain undetermined.…”

Section: Discussionmentioning

confidence: 99%

“…The identity of the responsible autoantigens in Brunsting–Perry type BP is still controversial. A patient reported with this constellation of findings had immunoglobulin (Ig)G autoantibodies directed to anchoring fibrils below the lamina densa by pre‐embedding immunogold electron microscopy . Moreover, cases of the patients with the features of Brunsting–Perry type BP and autoantibodies directed to type VII collagen have been reported .…”

Section: Introductionmentioning

confidence: 97%

Case with Brunsting–Perry‐like localized subepidermal blister formations and immunoglobulin G antibodies against unidentified basement membrane zone antigen

Sato-Shibuya

Dainichi

Egawa

et al. 2015

The Journal of Dermatology

View full text Add to dashboard Cite

Brunsting-Perry type bullous pemphigoid is defined by the blister formation limited to the head and neck, and autoantibodies to type VII collagen are detected in several cases. However, the pathomechanisms and autoantigens in this condition remain unknown. We report a 20-year-old female patient with a more than 2-year history of recurrent tense blisters localized on the face with no distinct atrophic scar formation. The patient had neither extensive sun exposure nor a history suggestive of contact dermatitis. Oral betamethasone was effective on the skin lesions. Histopathology revealed subepidermal blister formation with dermal infiltrates of neutrophils. Although direct and indirect immunofluorescence tests detected immunoglobulin G antibodies to the basement membrane zone (BMZ), no known dermal or epidermal autoantigens were detected in immunoblot analyses. Therefore, this case may be a rare variant of Brunsting-Perry type localized bullous pemphigoid with autoantibodies to an undetermined BMZ antigen.

show abstract

“…Skin In 25-30% of patients 19 Localized erythematous plaque near affected mucosal surfaces, generalized bullous eruption particularly on the head and upper body, or vegetating lesions occasionally 102,103 Scarring, cicatricial alopecia on the scalp 104,105 Oral cavity In over 85% of patients Desquamative gingivitis, erosions, ulceration ( Fig. 1) Pseudomembrane Vesicles rarely seen (Fig.…”

Section: Sites Involved Presentations and Potential Complicationsmentioning

confidence: 99%

Mucous Membrane Pemphigoid

Kirtschig¹,

Wojnarowska²

SpringerReference

View full text Add to dashboard Cite

Localized Cicatricial Pemphigoid of the Brunsting-Perry Type With Transition Into Disseminated Cicatricial Pemphigoid

Cited by 29 publications

References 39 publications

Heterogeneity of Brunsting-Perry type pemphigoid: A case showing blister formation at the lamina lucida, immune deposition beneath the lamina densa and autoantibodies against the 290-kD polypeptide along the lamina densa

Heterogeneity of Brunsting-Perry type pemphigoid: A case showing blister formation at the lamina lucida, immune deposition beneath the lamina densa and autoantibodies against the 290-kD polypeptide along the lamina densa

Case with Brunsting–Perry‐like localized subepidermal blister formations and immunoglobulin G antibodies against unidentified basement membrane zone antigen

Mucous Membrane Pemphigoid

Contact Info

Product

Resources

About