Pemphigus herpetiformis with autoantibodies to desmocollins 1, 2 and 3

Tay, Yuxin Evelyn; Lee, S. S. J.; Y, Lim

doi:10.1111/ced.13355

Cited by 3 publications

(8 citation statements)

References 7 publications

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“…Dsg1 was usually the target (32%), less commonly Dsg3 (7%) and Dsg1 and 3 (4%). Anti‐Dscs were also detected in a few cases, showing a variable combination of anti‐Dsg1 and 3 with anti‐Dsc 1, 2, and 3 (Table ) …”

Section: Resultsmentioning

confidence: 99%

“…The initial search yielded 371 publications: 284 from the PubMed database and 87 from the Ovid Medline database. We identified 96 publications (88 from PubMed and 63 from Ovid Medline [55 duplicates between the two databases]) reporting 158 individual cases that fulfilled the inclusion criteria. We also report an additional three unpublished cases diagnosed at our institution.…”

Section: Resultsmentioning

confidence: 99%

“…Both intercellular IgG and IgA deposits with/without C3 were mentioned for seven patients with pemphigus herptiformis; however, other cases of pemphigus have been described as IgG/ Table 3). [20][21][22][23][24][25][26][27][28][29][30][31][32] Association with other diseases As shown in Table 1, PH is seldom associated with other diseases, but its most frequent reported association was with other immunobullous disorders, especially other types of pemphigus (8%). Initially, PH was thought to be an unusual clinical and pathologic presentation of classical pemphigus (pemphigus vulgaris or pemphigus foliaceus) that could precede or occur during the course of these diseases.…”

Section: Immunologic Findingsmentioning

confidence: 99%

“…The pathophysiologic characteristics of PH are unclear. Some patients show immunoreactivity not only to desmogleins (Dsgs), as in classic forms of pemphigus, but also to desmocollins (Dscs) . Many mechanisms by which autoantibodies produce characteristic skin lesions of PH have been proposed, including autoantibody‐augmented signaling pathways that lead to increases in proinflammatory cytokines and chemokines and to subsequent epitope spreading …”

Section: Introductionmentioning

confidence: 99%

“…Some patients show immunoreactivity not only to desmogleins (Dsgs), as in classic forms of pemphigus, but also to desmocollins (Dscs). [20][21][22][23][24][25][26][27][28][29][30][31][32] Many mechanisms by which autoantibodies produce characteristic skin lesions of PH have been proposed, including autoantibody-augmented signaling pathways that lead to increases in proinflammatory cytokines and chemokines and to subsequent epitope spreading. 14 Pemphigus herpetiformis generally has a benign course, and most cases respond well to treatment with systemic corticosteroids or dapsone (or other sulfones), although other immunosuppressants may sometimes be required.…”

Section: Introductionmentioning

confidence: 99%

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Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

2019

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Pemphigus herpetiformis (PH), a rare type of pemphigus, is characterized by immunologic findings consistent with pemphigus but with a unique clinical and pathologic presentation. PH was first described as resembling dermatitis herpetiformis clinically, but because of its variable presentation, it can also resemble linear immunoglobulin A bullous dermatosis and bullous pemphigoid. We reviewed reported cases to analyze the most frequent clinical, pathologic, and immunologic characteristics and to propose corresponding diagnostic criteria. Through a comprehensive review of Medline and PubMed databases, 96 publications and 158 cases were identified. After reviewing the reported characteristics of PH, we suggest the following diagnostic criteria: Clinical: 1) pruritic herpetiform intact blisters with/without erosions; and/or 2) pruritic annular or urticarial erythematous plaques with/without erosions; Pathologic: 1) intraepidermal eosinophils or neutrophils, or both; and/or 2) intraepidermal split with/without acantholysis; Immunologic: 1) direct immunofluorescence showing immunoglobulin G with/without C3 intercellular deposits; and/or 2) indirect immunofluorescence showing immunoglobulin G to epithelial cell surface; and/or 3) detection of serum autoantibodies against desmogleins (1,3) or desmocollins (1,2,3), or both. Diagnosis requires one clinical, one pathologic, and one immunologic feature. We also report three new cases diagnosed at our institution to demonstrate the applicability of the suggested criteria.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Immunologic Findingsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

2019

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Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

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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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Pemphigus herpetiformis with autoantibodies to desmocollins 1, 2 and 3

Abstract: Click https://www.wileyhealthlearning.com/ced.aspx for the corresponding questions to this CME article.

Cited by 3 publications

References 7 publications

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

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