Three Cases of Transition from Pemphigus vulgaris to Pemphigus foliaceus Confirmed by Desmoglein ELISA

Ng, Patricia Pei‐Lin; Thng, Steven Tien Guan

doi:10.1159/000084757

Cited by 16 publications

(19 citation statements)

References 24 publications

(46 reference statements)

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“…The patient displayed the mucocutaneous PV phenotype when both DSG-1 and DSG-3 autoantibodies were expressed, while he displayed the PF phenotype when only the DSG-1 autoantibody was present. The findings in the current case are consistent with the majority of prior case reports which have found dramatic changes in DSG-1 and DSG-3 autoantibody levels upon transition between pemphigus types [3][4][5][6][7] . The occurrence of transition between pemphigus subtypes in the literature is infrequent, with less than 30 cases identified of PV to PF transition and even fewer cases of PF to PV transition [3][4][5][6][7] .…”

Section: Abstract Introductionsupporting

confidence: 92%

“…This concept is demonstrated in rare cases of transition between pemphigus subtypes. Diagnosis of transition is made on the basis of changes in classic phenotypic expression in conjugation with changes in immunofluorescence and/or antidesmoglein ELISA [3][4][5][6][7] . In this report, we describe a rare case of transition from PV to PF following rituximab therapy confirmed serologically by antidesmoglein ELISA.…”

Section: Abstract Introductionmentioning

confidence: 99%

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A Rare Transition from Pemphigus Vulgaris to Pemphigus Foliaceus Following Rituximab Therapy Confirmed by Antidesmoglein ELISA

2017

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Pemphigus defines a group of blistering diseases characterized by autoimmunity against intraepidermal adhesion proteins and subsequent flaccid blister formation, two major subtypes of which are pemphigus foliaceus (PF) and pemphigus vulgaris (PV). Of these, PF is typically more benign and characterized by IgG autoantibodies against desmoglein-1 (DSG-1) with exclusive cutaneous involvement. In contrast, PV is typically more severe and characterized by IgG autoantibodies against desmoglein-3 (DSG-3) in the mucosal predominant form and against both DSG-3 and DSG-1 in the mucocutaneous form 1 .July 2017 Volume I Issue I Copyright 2017 The National Society for Cutaneous MedicineThe phenotypic expression of pemphigus subtypes is mediated by differences in desmoglein (DSG) autoantibody profiles, a concept that is demonstrated in rare cases of transition between pemphigus subtypes. Diagnosis of transition is made on the basis of changes in classic phenotypic expression in conjugation with changes in immunofluorescence and/or antidesmoglein ELISA.We report a 54-year-old male with a history of severe mucocutaenous pemphigus vulgaris (PV) brought into remission by treatment with systemic steroids and adjuvant rituximab infusions who re-presented approximately 1 year following steroid discontinuation with new crusted erosions covering the upper trunk and proximal extremities and notable absence of mucosal involvement. Antidesmoglein ELISA profile revealed an isolated rise in DSG-1 antibodies. Previous antidesmoglein ELISA profiles showed dual elevation in both DSG-1 and DSG-3 antibodies during active PV and absence of both DSG-1 and DSG-3 during disease remission.The current case describes a rare transition from PV to pemphigus foliaceus (PF) following rituximab therapy as suspected clinically by classic phenotypic expression and confirmed serologically by antidesmoglein ELISA. This is only the second reported case of pemphigus transition following rituximab adjuvant therapy, and there are less than thirty cases of PV to PF transition reported in the literature. There is currently not a satisfactory explanation for the autoantibody shifting that is observed in transition between pemphigus types. ABSTRACT INTRODUCTION

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Section: Abstract Introductionsupporting

confidence: 92%

Section: Abstract Introductionmentioning

confidence: 99%

A Rare Transition from Pemphigus Vulgaris to Pemphigus Foliaceus Following Rituximab Therapy Confirmed by Antidesmoglein ELISA

2017

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“…Therefore, the assessment of early time points in PV patients will be necessary to formally determine the relation between serum sCD40L and pemphigus. In addition, since some PV patients show transition to pemphigus foliaceus in the disease course [25], it may also be interesting to elucidate the participation of sCD40L in the transition of antibodies from Dsg3 to Dsg1.…”

Section: Discussionmentioning

confidence: 99%

Increased Serum Levels of Circulating CD40 Ligand in Patients with Bullous Pemphigoid: Preliminary Results

Watanabe

Ishiura²,

Nakashima³

et al. 2007

Dermatology

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Background: Autoimmune bullous diseases are characterized by autoantibodies against specific adhesion molecules of the skin and/or mucous membrane. While these autoantibodies are known to play a primary role in the disease manifestation, it remains unknown how disease-specific autoreactive B cells and autoantibodies are induced. Recent studies have indicated the importance of the CD40 and CD40 ligand (CD40L) receptor-ligand pair in the immunopathogenesis of autoimmune diseases. CD40L circulates in soluble form, and some reports suggest that serum soluble CD40L (sCD40L) levels are increased in various autoimmune diseases. Objectives: To determine serum sCD40L levels in patients with pemphigus vulgaris (PV) and bullous pemphigoid (BP), and to determine their correlation with clinical findings and laboratory findings. Patients and Methods: Sera from 10 PV patients, 35 BP patients and 12 normal controls were subjected to ELISA assays to measure serum levels of sCD40L, anti-desmoglein-3 antibody and anti-BP180 antibody. Results and Conclusions: Circulating sCD40L levels were significantly elevated in BP patients, but not in PV patients. Serum sCD40L levels increased in the early stage of disease onset and recurrence in BP patients. In conclusion, circulating sCD40L levels may be a useful marker for early activation of autoimmune diathesis and, furthermore, an effective therapeutic target in patients with BP.

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“…Pemphigus vulgaris (PV) is caused by autoantibodies against Dsg3, while pemphigus foliaceus (PF), a usually less severe variant, is caused by autoantibodies against Dsg1 [1]. Moreover, cases of transition from PV to PF and PF to PV confirmed by a change in Dsg 1 and 3 autoantibody profile by enzyme-linked immunosorbent assay (ELISA) have very rarely been reported [2]. On the other hand, a crucial role of autoreactive T cells in the induction and regulation of autoantibody production in pemphigus has recently been suggested [3].…”

Section: Introductionmentioning

confidence: 99%

Treatment of Refractory Pemphigus with the Anti-CD20 Monoclonal Antibody (Rituximab)

et al. 2007

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Background: Pemphigus is a severe blistering disorder caused by autoantibodies to desmogleins 1 and 3. Because some patients with pemphigus never enter into remission, new immunosuppressants are warranted. Rituximab is a chimeric monoclonal antibody binding to the CD20 antigen on B cells, which proved to be effective in recalcitrant pemphigus. Objectives: To evaluate the efficacy and safety of rituximab in refractory pemphigus and to investigate its effects on the autoantibody profile. Patients and Methods: Six patients with recalcitrant pemphigus were treated. Rituximab was administered intravenously at a dosage of 375 mg/m² body surface once weekly for 4 weeks. Results: Three pemphigus foliaceus patients and 1 with mucocutaneous pemphigus vulgaris (PV) showed complete response over a follow-up period of up to 18 months. In one oral PV, control of the disease was achieved using pulse therapy with cyclophosphamide following rituximab withdrawal. In one PV with vegetating features, good improvement was obtained after 6 rituximab infusions. All patients tolerated the treatment well. Anti-desmoglein autoantibodies significantly decreased only in pemphigus foliaceus. Conclusions: This study highlights that rituximab is a valuable drug for refractory pemphigus, although the response of mucous membranes and cutaneous folds may be delayed.

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Three Cases of Transition from Pemphigus vulgaris to Pemphigus foliaceus Confirmed by Desmoglein ELISA

Cited by 16 publications

References 24 publications

A Rare Transition from Pemphigus Vulgaris to Pemphigus Foliaceus Following Rituximab Therapy Confirmed by Antidesmoglein ELISA

A Rare Transition from Pemphigus Vulgaris to Pemphigus Foliaceus Following Rituximab Therapy Confirmed by Antidesmoglein ELISA

Increased Serum Levels of Circulating CD40 Ligand in Patients with Bullous Pemphigoid: Preliminary Results

Treatment of Refractory Pemphigus with the Anti-CD20 Monoclonal Antibody (Rituximab)

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