Pemphigus herpetiformis with IgG autoantibodies to desmoglein 1 and desmocollin 1

On, Hye Rang; Hashimoto, Takashi; Kim, Soo Chan

doi:10.1111/bjd.13419

Cited by 15 publications

(10 citation statements)

References 10 publications

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“…Anti‐Dsc antibodies exist in patients with paraneoplastic or atypical pemphigus, including PH and pemphigus vegetans . There have been previous reports of PH with anti‐Dsc antibodies . Like our patient, one case reported the presence of anti‐Dsc1 antibodies without anti‐Dsg antibodies in PH .…”

Section: Discussionsupporting

confidence: 60%

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Case of shift from linear immunoglobulin A bullous dermatosis to pemphigus herpetiformis for a short period of time

Koga

Ishii

Hashimoto

et al. 2016

The Journal of Dermatology

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Pemphigus herpetiformis (PH) is a rare variant of pemphigus characterized by erythemas and vesicles, tending to present with annular-shaped lesions. Immunologically, immunoglobulin (Ig)G deposition at the keratinocyte cell surfaces is observed. Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disease with linear IgA deposits at the epidermal basement membrane zone (BMZ). The annular-shaped skin lesions in PH mimic clinical manifestation of other autoimmune bullous diseases, including LABD, although PH and LABD have different immunological and histopathological features. Herein, we report the first case of a shift from LABD to PH. A 70-year-old Japanese man presented annular erythemas surrounded by vesicles on the trunk and extremities. Histopathological examination revealed subepidermal bullae and eosinophilic spongiosis. Direct immunofluorescence demonstrated linear IgA deposits at the epidermal BMZ. Immunoblot analyses of normal human epidermal and dermal extracts, supernatant of HaCaT cells, recombinant proteins of BP180 NC16a and C-terminal domains, and purified laminin-332 showed no reactivity for either IgG or IgA. IgG chemiluminescent enzyme immunoassays for desmogleins 1 and 3, and BP180 were all negative. These findings led to the diagnosis of sole LABD. Although oral prednisolone temporarily improved the skin lesions, annular erythema without vesicles remained. A new skin biopsy revealed subcorneal pustules with eosinophils, but no subepidermal bullae. Direct immunofluorescence revealed IgG and C3 deposition at the keratinocyte cell surfaces. IgG enzyme-linked immunosorbent assay for mammalian desmocollins 1-3 revealed desmocollin 1 reactivity. Based on these findings, we made a diagnosis of sole PH.

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Section: Discussionsupporting

confidence: 60%

“…5 There have been previous reports of PH with anti-Dsc antibodies. [10][11][12][13][14] Like our patient, one case reported the presence of anti-Dsc1 antibodies without anti-Dsg antibodies in PH. 11 These findings suggest that PH is a phenotype for anti-Dsc1 antibodies.…”

Section: Discussionsupporting

confidence: 57%

Case of shift from linear immunoglobulin A bullous dermatosis to pemphigus herpetiformis for a short period of time

Koga

Ishii

Hashimoto

et al. 2016

The Journal of Dermatology

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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 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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Autoimmune Bullous Diseases

Beek

Schmidt

2019

Harper's Textbook of Pediatric Dermatology

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Pemphigus herpetiformis with IgG autoantibodies to desmoglein 1 and desmocollin 1

Cited by 15 publications

References 10 publications

Case of shift from linear immunoglobulin A bullous dermatosis to pemphigus herpetiformis for a short period of time

Case of shift from linear immunoglobulin A bullous dermatosis to pemphigus herpetiformis for a short period of time

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

Autoimmune Bullous Diseases

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