Atypical Pemphigus with Concomitant IgG and IgA Anti-Intercellular Autoantibodies Associated with Monoclonal IgA Gammopathy

Ohno, Haruhiko; Miyagawa, Shuji; Hashimoto, Toshihiro; Nakagawa, Akemi; Watanabe, Kazuhiro; Nishikawa, Takeji; Shirai, Toshihiko

doi:10.1159/000246948

Cited by 9 publications

(6 citation statements)

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“…The histological findings showed neutrophilic spongiosis mainly without eosinophils in the subcorneal and granular layers of the epidermis. 24,25 IgA antibody was not detected in this case by either direct or indirect immunofluorescence, but there is a possibility that the IgA antibody titre was too low to be detectable. 18,21 On the other hand, most herpetiform pemphigus cases showed autoantibodies to desmoglein 1, 14,22 which is known to be specific for PF.…”

Section: Discussionmentioning

confidence: 66%

“…20 Although only IgA antiintercellular antibody is usually detected in sera from both types of IgA pemphigus, 20 rare cases with both IgA and IgG anti-intercellular antibodies have been reported. 24,25 IgA antibody was not detected in this case by either direct or indirect immunofluorescence, but there is a possibility that the IgA antibody titre was too low to be detectable.…”

Section: Discussionmentioning

confidence: 66%

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Pemphigus with features of both vulgaris and foliaceus variants, associated with antibodies to 160 and 130 kDa antigens

Izumi

Seishima

Satoh

et al. 1998

British Journal of Dermatology

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We report an unusual variant of pemphigus in a 44-year-old man. He presented with scaly and crusted erosions associated with pruritic vesicles and erythema mainly on the chest, abdomen, back and face. Histology showed acantholysis with neutrophilic spongiosis in the granular layer and subcorneal region of the epidermis. Intercellular IgG in the epidermis was positive on direct immunofluorescence. Indirect immunofluorescence showed intercellular antibodies at a titre of 1 : 2 in the suprabasal epidermis. Circulating autoantibodies to 130 kDa and 160 kDa antigens were detected in the patient's serum by immunoblot analysis using epidermal extracts. These two antibodies eluted from individual nitrocellulose membranes reacted with the intercellular space in the epidermis on indirect immunofluorescence. This observation suggests that these antibodies correspond to desmogleins 3 and 1, respectively. The clinical symptoms almost completely disappeared after 28 days treatment with oral prednisolone (30 mg/day), leaving brown pigmented flecks on the lesional sites. These findings suggest that this patient's pemphigus has features of both the vulgaris and foliaceus variants, with antibodies against desmogleins 3 and 1.

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Section: Discussionmentioning

confidence: 66%

Section: Discussionmentioning

confidence: 66%

Pemphigus with features of both vulgaris and foliaceus variants, associated with antibodies to 160 and 130 kDa antigens

Izumi

Seishima

Satoh

et al. 1998

British Journal of Dermatology

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“…Th e development of skin lesions in these diseases is causally related to IgA immunoglobulin binding to various skin antigens [8,9]. Minor diff erences in the clinical presentation between these syndromes are probably due to diff erent affi nities of the antigenic determinant of the deposed IgA immunoglobulin [10,11].…”

Section: Discussionmentioning

confidence: 99%

IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen

Adam

Krejčí

Pour

et al. 2010

Wien Klin Wochenschr

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Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year-old woman with IgA pemphigus and monoclonal gammopathy of unknown significance had been treated unsuccessfully with cyclophosphamide/dexamethasone and then with rituximab. When the monoclonal gammopathy progressed to multiple myeloma, the patient received treatment with cyclophosphamide/doxorubicin/dexamethasone but there was no clinical response. Second-line therapy with a thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with a combination regimen that included bortezomib, cyclophosphamide and dexamethasone resulted in complete and durable remission of multiple myeloma and IgA pemphigus. This suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy.

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“…It is likely that, as with other neutrophilic dermatoses, IgA pemphigus may sometimes be associated with monoclonal gammapathy (16,24). Although extensive laboratory studies did not reveal evidence of gammopathy in our patients it seems prudent to evaluate all patients with IgA pemphigus for the presence of this condition and the associated haematological myeloproliferative disorder.…”

Section: Discussionmentioning

confidence: 66%

IgA Pemphigus: The First Two Scandinavian Cases

Gniadecki¹,

Bygum²,

Clemmensen³

et al. 2002

Acta Dermato-Venereologica

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IgA pemphigus is a rare neutrophilic acantholytic skin disorder with only approximately 70 cases reported in the indexed literature to date. Here we describe two patients with IgA pemphigus (subcorneal pustular dermatosis type and intraepithelial neutrophilic type) that to our knowledge are the first Scandinavian patients with this disease. Initially, both patients were misdiagnosed as subcorneal pustular dermatosis of Sneddon and Wilkinson and only subsequent careful immunofluorescence studies (in one case with confocal laser scanning microscopy) led to the correct diagnosis. Apart from the expected IgA depositions on epidermal cell surfaces, both patients demonstrated some degree of intercellular IgG-specific immunofluorescence. No circulating IgA autoantibodies were detected. One patient was treated with the standard regime comprising dapsone and prednisolone, whereas in the other case a novel methotrexate and prednisolone combination treatment showed marked clinical efficacy.

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Atypical Pemphigus with Concomitant IgG and IgA Anti-Intercellular Autoantibodies Associated with Monoclonal IgA Gammopathy

Cited by 9 publications

References 0 publications

Pemphigus with features of both vulgaris and foliaceus variants, associated with antibodies to 160 and 130 kDa antigens

Pemphigus with features of both vulgaris and foliaceus variants, associated with antibodies to 160 and 130 kDa antigens

IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen

IgA Pemphigus: The First Two Scandinavian Cases

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