A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading

Fairley, Janet A.; Woodley, David T.; Chen, Mei; Giudice, George J.; Lin, Mong-Shang

doi:10.1016/j.jaad.2003.12.033

Cited by 50 publications

(28 citation statements)

References 36 publications

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“…A patient with both BP and EBA was reported by Fairley et al [9] (2004). This patient exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease.…”

Section: Discussionmentioning

confidence: 99%

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients

Dias

Vilar

Bento

et al. 2018

Skin Appendage Disord

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Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare variant of cicatricial pemphigoid, characterized by bullous lesions limited to the head, neck, scalp, and upper trunk with mild or no mucosal involvement. We report 2 cases of BPP cicatricial alopecia with histopathology of subepidermal blister formation, different clinical presentation, and different salt-split test results. One patient had features of bullous pemphigoid (BP) with important oral mucosal involvement (not yet reported in the literature), and the second patient had typical features of epidermolysis bullosa acquisita (EBA). The secondary cicatricial alopecia may be due to different antigens associated with either BP or EBA. The phenomenon of epitope spreading could explain the association between 2 distinctive bullous diseases in the same patient, justifying the divergent findings of the immunofluorescence. The specific target antigen of BPP is yet to be defined.

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Section: Discussionmentioning

confidence: 99%

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients

Dias

Vilar

Bento

et al. 2018

Skin Appendage Disord

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“…Ab diversification to epitopes of BP180 and BP230 [108,130] Clinical study Intramolecular and intermolecular Ab spread from collagen XVII to collagen VII [109] Clinical report Intermolecular Bullous pemphigoid develops after pemphigus foliaceus [13,14] Clinical report Intermolecular Cardiomyopathy…”

Section: Diseasementioning

confidence: 99%

B cell epitope spreading: Mechanisms and contribution to autoimmune diseases

Cornaby¹,

Gibbons²,

Mayhew³

et al. 2015

Immunology Letters

149

108

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“…In autoimmune diseases, it is well known that autoantibodies of patients in the later disease stages are more likely to react with multiple sites of autoantigens than those of patients in early stages of the disease. This is known as ‘epitope spreading’ [30,31,32]. However, in our case, IgG autoantibodies against Col17 could already be detected from the very early stages when the patient initially presented at our clinic.…”

Section: Discussionmentioning

confidence: 60%

A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation

Goto-Ohguchi

Nishie²,

Akiyama³

et al. 2008

Dermatology

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Anti-p200 pemphigoid is a recently described autoimmune blistering skin disease that is characterized by the presence of autoantibodies against an unidentified 200-kDa dermal autoantigen. Most of the previous cases have been successfully treated using mild-to-moderate immunosuppressive therapies, which resulted in a good prognosis. We report here a severe and refractory case of anti-p200 pemphigoid that developed in a 53-year-old woman, in which blisters led to multiple skin ulcers, followed by severe scar formation. In the present case, methylprednisolone pulse therapy was effective enough to reduce the disease activity.

show abstract

A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading

Cited by 50 publications

References 36 publications

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients

B cell epitope spreading: Mechanisms and contribution to autoimmune diseases

A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation

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