Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3

Kozłowska, Anna; Hashimoto, Takashi; Jarzabek-Chorzelska, M; Amagai, A.; Nagata, Yoshiko; Strasz, Z.; Jabłońska, Stefania

doi:10.1067/mjd.2003.23

Cited by 68 publications

(69 citation statements)

References 20 publications

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“…was reported as PH with IgG and IgA antibodies to Dsgl (14). Specific IgG and IgA ELISA disclosed high titres of IgA and IgG anti-Dsg I antibodies, and no reactivity to Dsg3.…”

Section: Discussionmentioning

confidence: 94%

IgA/IgG Pemphigus: A New Atypical Subset of Pemphigus?

Kowaléwski¹,

Hashimoto²,

Amagai³

et al. 2006

Acta Derm Venereol

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“…was reported as PH with IgG and IgA antibodies to Dsgl (14). Specific IgG and IgA ELISA disclosed high titres of IgA and IgG anti-Dsg I antibodies, and no reactivity to Dsg3.…”

Section: Discussionmentioning

confidence: 94%

IgA/IgG Pemphigus: A New Atypical Subset of Pemphigus?

Kowaléwski¹,

Hashimoto²,

Amagai³

et al. 2006

Acta Derm Venereol

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“…[7,8] Kozlowska et al reported a case of IgG/IgA pemphigus with clinical features of pemphigus herpetiformis and IgG and IgA reactivity with Dsg1 with additional IgG reactivity to Dsc1. [9] Pemphigus herpetiformis show IgG reactivity frequently with Dsg1 and occasionally with Dsg3. [10] Interestingly, all reported cases of IgG/IgA pemphigus (including the present case) with IgG and IgA reactivity exclusively with Dsg1 clinically behaved like pemphigus herpetiformis.…”

Section: Discussion Discussionmentioning

confidence: 99%

IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone

Kanwar¹,

Vinay²,

Saikia³

et al. 2014

Indian J Dermatol Venereol Leprol

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IgG/IgA pemphigus is an extremely rare subset of pemphigus, showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes. Herein, we describe a unique case of IgG/IgA pemphigus with clinical features of edematous erythema and peripheral vesiculopustules. Histopathology showed the presence of subcorneal pustules and acantholytic blisters in the mid-epidermis with neutrophilic infiltration and eosinophilic spongiosis. Direct immunofluorescence of perilesional skin showed both IgG and IgA deposits to keratinocyte cell surfaces and unusual granular deposits of IgG, IgM, and C3 along basement membrane zone. On enzyme linked immunosorbent assay , the auto-antibodies were found to be reactive to desmoglein 1 antigen. Various clinical, histopathological, and immunological findings in our case overlapped with the features of IgA pemphigus, pemphigus herpetiformis, and pemphigus foliaceus. These findings indicate that IgG/IgA pemphigus may be a transitional form between IgA pemphigus and pemphigus herpetiformis, and thus provides insight into the pathogenicity of this rare disorder.

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“…We present a case of anti-Dsc3 PV, illustrated in a patient with typical mucosal disease. Previously, atypical or paraneoplastic pemphigus patients with human Dsc3 immunoreactivity have been described, 17,[22][23][24] although autoantibodies in these patients reacted with multiple skin antigens, and pathogenicity against Dsc3 was not demonstrated, raising the possibility that the Dsc3 autoantibodies may not have caused disease, but instead arose secondarily as a result of epidermal damage. Figure 2A and unpublished data in- bearing an E tag were produced in a baculovirus expression system.…”

Section: Discussionmentioning

confidence: 99%

Autoimmunity to Desmocollin 3 in Pemphigus Vulgaris

Mao

Nagler

Farber

et al. 2010

The American Journal of Pathology

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Pemphigus vulgaris is a blistering disease associated with autoantibodies to the desmosomal adhesion protein, desmoglein 3. Genetic deficiency of desmoglein 3 in mice mimics autoimmunity to desmoglein 3 in pemphigus vulgaris, with mucosal-dominant blistering in the suprabasal layer of the epidermis. Mice with an epidermal-specific deletion of desmocollin 3, the other major desmosomal cadherin isoform expressed in the basal epidermis, develop suprabasal blisters in skin that are histologically identical to those observed in pemphigus vulgaris, suggesting that desmocollin 3 might be a target of autoantibodies in some pemphigus vulgaris patients. We now demonstrate that desmocollin 3 is an autoantigen in pemphigus vulgaris, illustrated in a patient with mucosaldominant blistering. Six of 38 pemphigus vulgaris and one of 85 normal serum samples immunoprecipitate desmocollin 3 (P ‫؍‬ 0.003). Incubation of patient IgG with human keratinocytes causes loss of intercellular adhesion, and adsorption with recombinant desmocollin 3 specifically prevents this pathogenic effect. Additionally, anti-desmocollin 3 sera cause loss of keratinocyte cell surface desmocollin 3, but not desmoglein 3 by immunofluorescence , indicating distinct cellular pathogenic effects in anti-desmocollin and antidesmoglein pemphigus , despite their identical clinical presentations. These data demonstrate that desmocollin 3 is a pathogenic autoantigen in pemphigus vulgaris and suggest that pemphigus vulgaris is a histological reaction pattern that may result from autoimmunity to desmoglein 3, desmocol-

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Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3

Cited by 68 publications

References 20 publications

IgA/IgG Pemphigus: A New Atypical Subset of Pemphigus?

IgA/IgG Pemphigus: A New Atypical Subset of Pemphigus?

IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone

Autoimmunity to Desmocollin 3 in Pemphigus Vulgaris

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