Pemphigus vegetans – immunopathological findings in a rare variant of pemphigus vulgaris

Itzinger-Monshi, Babak; Marker, Michael; Feichtinger, Hans G.; Schmid, G; Kriehuber, Ernst; Födinger, Dagmar; Rappersberger, Klemens

doi:10.1111/j.1610-0387.2009.07214.x

Cited by 21 publications

(34 citation statements)

References 10 publications

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“…21 For pemphigus vegetans, azathioprine seems to work in individual cases. 22 For treatment of refractory patients, disease control has been reported under the corticosteroid/azathioprine regimen using additionally photopheresis or retinoids. 23,24 In paraneoplastic pemphigus, therapy for the underlying malignancy is essential.…”

Section: Azathioprine In Pemphigusmentioning

confidence: 99%

Azathioprine in the Treatment of Autoimmune Blistering Diseases

Meyer¹,

Beissert²

2011

Dermatologic Clinics

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Section: Azathioprine In Pemphigusmentioning

confidence: 99%

Azathioprine in the Treatment of Autoimmune Blistering Diseases

Meyer¹,

Beissert²

2011

Dermatologic Clinics

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“…To compare our patient's antibody profile with autoimmunological findings of previously described patients suffering from PVeg, we reviewed the Anglophone literature from December 1988 to January 2017, considering all available full-text publications providing information on mucous membranes and/or skin involvement and autoantibody levels (at least anti-Dsg1 and/or anti-Dsg3 reactivity; Table 1) [2, 3, 4, 5, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34]. …”

Section: Review Of the Literature And Discussionmentioning

confidence: 99%

Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature

et al. 2017

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Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger’s tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.

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“…In pemphigus vulgaris, cellcell adhesion is disrupted at the basal layer while the basement membrane zone is intact, resulting in the 'row of tombstones' appearance [36,37]. Pemphigus vegetans shows epidermal hyperplasia and eosinophilic/neutrophilic pustules in addition to cleft formation [38]. Pemphigus herpetiformis shows faint acantholysis and eosinophilic spongiosis [10].…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

Diagnosis and Treatment of Pemphigus

2012

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Pemphigus is an autoimmune bullous disease, in which autoantibodies react with the cell-cell adhesion structures, desmosomes, causing blisters and erosions on the oral mucosa and skin. Pemphigus is divided into two major subtypes: pemphigus vulgaris and pemphigus foliaceus. Oral corticosteroids are the primary treatment modality for pemphigus, while other therapeutic options, such as steroid pulse therapy, immunosuppressants, intravenous immunoglobulins, plasmapheresis and anti-CD20 monoclonal antibody therapy, are occasionally employed. Immunosuppressants used to treat pemphigus include azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil and mizoribine. In this review, we summarize the current concepts of immunotherapy for the treatment of pemphigus in the Japanese population.

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Pemphigus vegetans – immunopathological findings in a rare variant of pemphigus vulgaris

Cited by 21 publications

References 10 publications

Azathioprine in the Treatment of Autoimmune Blistering Diseases

Azathioprine in the Treatment of Autoimmune Blistering Diseases

Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature

Diagnosis and Treatment of Pemphigus

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