Successful monotherapy of pemphigus vegetans with minocycline and nicotinamide

Köckritz, Amelie von; Ständer, Sonja; Zeidler, Claudia; Metze, Dieter; Luger, Thomas A.; Bonsmann, Gisela

doi:10.1111/jdv.13779

Cited by 12 publications

(4 citation statements)

References 20 publications

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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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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, a rare clinical variant of PV which represents 1-2% of all pemphigus cases (von Köckritz et al, 2017). Due to it's rarity, only few papers in the literature are specifically devoted to it with most of them are case reports (Mergler et al, 2017;Cuellar et al, 2020;Liu et al, 2020;Verma et al, 2020).…”

Section: Pemphigus Vegetansmentioning

confidence: 99%

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Lim

Bohelay

Hanakawa

et al. 2022

Front. Mol. Biosci.

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Pemphigus represents a group of rare and severe autoimmune intra-epidermal blistering diseases affecting the skin and mucous membranes. These painful and debilitating diseases are driven by the production of autoantibodies that are mainly directed against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1). The search to define underlying triggers for anti-Dsg-antibody production has revealed genetic, environmental, and possible vaccine-driven factors, but our knowledge of the processes underlying disease initiation and pathology remains incomplete. Recent studies point to an important role of T cells in supporting auto-antibody production; yet the involvement of the myeloid compartment remains unexplored. Clinical management of pemphigus is beginning to move away from broad-spectrum immunosuppression and towards B-cell-targeted therapies, which reduce many patients’ symptoms but can have significant side effects. Here, we review the latest developments in our understanding of the predisposing factors/conditions of pemphigus, the underlying pathogenic mechanisms, and new and emerging therapies to treat these devastating diseases.

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“…Congenital leukaemia is rare with <200 cases described in the literature. 1 Skin is the most frequent infiltration location, occurring in around 25-30% of all cases. 2 Cutaneous manifestations of leukaemia mostly present the so-called blueberry muffin baby appearance as multiple, firm, freely movable violaceous, blue or red nodules approximately 1-2.5 cm in diameter.…”

Section: Disseminated Cobblestone-like Skin Lumps In a Newbornmentioning

confidence: 99%