Unique herpetiform bullous dermatosis with IgG antibodies to desmocollins 1/3 and LAD-1

Ueda, Akihiro; Ishii, Norito; Teye, Kwesi; Dainichi, Teruki; Ohyama, Bungo; Hamada, Takahiro; Tsuruta, Daisuke; Kamioka, Naomi; Mitsui, Konomi; Hosaka, Hiroo; Soh, Inketsu; Nakada, Tokio; Hashimoto, Takashi

doi:10.1111/bjd.12398

Cited by 11 publications

(18 citation statements)

References 10 publications

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“…However, one unique case of herpetiform bullous dermatosis with IgG antibodies to Dsc1 and Dsc3 as well as LAD-1 was reported. 9 Desmocollin 1 IgG ELISA was positive during the disease course, although OD levels were not parallel with disease activity. This suggests the presence of IgG antibodies to other unknown components of the keratinocyte CS because CLEIA or ELISA for Dsg1, Dsg3 and Dsc1-3, that are known as autoantigens for pemphigus, showed no reactivity at several points during the disease course, other than Dsc1.…”

Section: Discussionmentioning

confidence: 89%

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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: Discussionmentioning

confidence: 89%

“…This case may suggest an interaction between LABD and PH, although no other reports have provided collaborating evidence so far. However, one unique case of herpetiform bullous dermatosis with IgG antibodies to Dsc1 and Dsc3 as well as LAD‐1 was reported …”

Section: Discussionmentioning

confidence: 99%

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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“…Conversely, IgA autoantibodies are far more common in apparently 'typical' IgG-mediated pemphigus vulgaris than generally assumed, as shown in two large studies. 20 In light of the chronicity of the patient's skin disorder (> 10 years), the unique autoantibody profile was probably related to epitope spreading. The lack of detection of epidermal intercellular deposits of IgA or IgG on direct immunofluorescence in our case is puzzling.…”

Section: Discussionmentioning

confidence: 99%

The expanding spectrum ofIgApemphigus: a case report and review of the literature

et al. 2014

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IgA pemphigus (IGAP) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD-type IGAP, we demonstrate the coexistence of IgA reactivity to both epidermal (desmocollins 2 and 3) and basement membrane-associated (BP180) proteins, suggesting the coexistence of atypical IGAP and linear IgA bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP, underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term 'IGAP spectrum'.

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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%

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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Unique herpetiform bullous dermatosis with IgG antibodies to desmocollins 1/3 and LAD-1

Cited by 11 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

The expanding spectrum ofIgApemphigus: a case report and review of the literature

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

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