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

Goto-Ohguchi, Yuka; Nishie, Wataru; Akiyama, Masashi; Tateishi, Y.; Aoyagi, Satoka; Tsuji-Abe, Yukiko; Sawamura, Daisuke; Ishii, Norito; Hashimoto, Takashi; Shimizu, Hiroshi

doi:10.1159/000182268

Cited by 16 publications

(10 citation statements)

References 67 publications

(60 reference statements)

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“…The autoantibodies mostly target two major components of the DEJ: BP230, also called BPAg1, an intracellular protein of the hemidesmosomal plaque, and BP180, or BPAg2, a transmembrane protein whose dominant epitope is located in the NC16A domain [3]. However, other antigens have been rarely reported in BP, such as in p200 pemphigoid [4]. The pathogenic role of BP180 antibodies in BP has been demonstrated [5].…”

Section: Introductionmentioning

confidence: 99%

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

Peufeilhoux¹,

Ingen‐Housz‐Oro²,

Hüe³

et al. 2012

Dermatology

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Background: The value of 230-kDa bullous pemphigoid antibody (BP230) enzyme-linked immunosorbent assay (ELISA) for the diagnosis of bullous pemphigoid (BP) was investigated, but in the immunological follow-up of the disease remains unknown. Objective: Evaluation of BP230 ELISA for diagnosis, follow-up and prediction of relapse in BP. Methods: Monocenter retrospective and prospective study. Patients with typical BP. Detection of autoantibodies by indirect immunofluorescence (IIF), BP180 and BP230 ELISA tests at diagnosis, during the treatment (disease control or failure) and at treatment stop (relapse or not 3 months after). Results: 74 patients were included. At diagnosis, BP230 ELISA sensitivity was lower than IIF and BP180 ELISA. Combining both ELISA added a weak gain of sensitivity. Both tests paralleled the clinical evolution, especially in case of disease control. At the end of the treatment, BP230 ELISA was not different in patients with or without relapse. Conclusion: In routine practice, BP230 ELISA does not seem to be a useful additional test in typical BP.

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Section: Introductionmentioning

confidence: 99%

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

Peufeilhoux¹,

Ingen‐Housz‐Oro²,

Hüe³

et al. 2012

Dermatology

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“…It was reported that approximately 25% of patients with anti‐laminin γ1 pemphigoid have reactivity against other autoantigens . Simultaneous autoimmune response to laminin γ1 and BP180 has been reported in four cases including our case, although other three cases did not have psoriasis . This phenomenon may be explained as intermolecular epitope spreading following the inflammatory damage to the BMZ .…”

Section: Discussionmentioning

confidence: 50%

“…2 Simultaneous autoimmune response to laminin c1 and BP180 has been reported in four cases including our case, although other three cases did not have psoriasis. [5][6][7] This phenomenon may be explained as intermolecular epitope spreading following the inflammatory damage to the BMZ. 2,8 Although the etiopathogenesis of psoriasis is unknown, an important role of tumor necrosis factor (TNF)-a-inducible plasmacytoid dendritic cells and T-helper 17 lymphocytes has been described.…”

Section: Discussionmentioning

confidence: 99%

Pemphigoid with antibodies to laminin γ1, BP180 and BP230, associated with psoriasis vulgaris: Successful disease control with cyclosporin

Imanishi

Tateishi

Imanishi

et al. 2015

The Journal of Dermatology

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Both anti-laminin γ1 pemphigoid and bullous pemphigoid are autoimmune subepidermal blistering diseases. The former is rare and characterized by autoantibodies to laminin γ1, a 200-kDa dermal protein, while the latter is common among the elderly and characterized by autoantibodies to BP180 and BP230, both of which are hemidesmosomal proteins. We experienced a 69-year-old Japanese male patient with blister formation secondary to erythrodermic psoriasis, which was successfully treated with cyclosporin. The histopathology of erythema corresponded with psoriasis and that of a blistering lesion showed infiltration of neutrophils and eosinophils in and around the subepidermal blisters. Patient immunoglobulin G antibodies labeled both the epidermal and dermal sides of 1 mol/L NaCl-split human skin by indirect immunofluorescent microscopy and recognized laminin γ1, BP180 and BP230 by immunoblotting. To the best of our knowledge, this is the first report of coexistence of psoriasis and atypical pemphigoid with these three autoantibodies.

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“…Recovery with scarring was occasionally reported in BP cases with IgG autoantibodies to LAD‐1, a case of anti‐laminin gamma‐1 (p200) pemphigoid, and a case of a blistering disease with IgG autoantibodies to BP180, BP230 and laminin‐332 …”

Section: Discussionmentioning

confidence: 99%

“…Recovery with scarring was occasionally reported in BP cases with IgG autoantibodies to LAD-1, 7 a case of anti-laminin gamma-1 (p200) pemphigoid, 8 disease with IgG autoantibodies to BP180, BP230 and laminin-332. 9 The clinical features in our patient mimicked classical EBA.…”

Section: Discussionmentioning

confidence: 99%

Refractory bullous pemphigoid leaving numerous milia during recovery

Uchida

Oiso

Koga

et al. 2014

The Journal of Dermatology

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Recovery with milia may occur in bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). Scarring commonly occurs in MMP and EBA. Here, we report a 62-year-old man patient with BP, who was left with numerous milia during recovery. The patient had immunoglobulin (Ig)G autoantibodies to the recombinant protein of the BP180-NC16a domain and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1). There are cases of BP with IgG autoantibodies to LAD-1 and/or the recombinant protein of BP180 C-terminal domain. Extensive milia formation during recovery may be associated with immunological predisposition and/or improper interaction between hemidesmosomes and the extracellular matrix components.

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

Cited by 16 publications

References 67 publications

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

Pemphigoid with antibodies to laminin γ1, BP180 and BP230, associated with psoriasis vulgaris: Successful disease control with cyclosporin

Refractory bullous pemphigoid leaving numerous milia during recovery

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