IgA pemphigus – Occurrence of anti‐Desmocollin 1 and anti‐Desmoglein 1 antibody reactivity in an individual patient

Kopp, Tamara; Sitaru, Cassian; Pieczkowski, Friederike; Schneeberger, Achim; Födinger, Dagmar; Zillikens, Detlef; Stingl, Georg; Karlhofer, Franz

doi:10.1111/j.1610-0387.2006.06166.x

Cited by 22 publications

(7 citation statements)

References 19 publications

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“…Some previous cases of IgA pemphigus showed IgA reactivity with the entire epidermis, despite the presence of subcorneal pustules. IgA antibodies in one case reacted to Dsc1–3, 2 while another case reacted to Dsc1 and Dsg1 3 . The findings in these cases are different from the unique clinical and histopathological features seen in our case, which showed small pustules in the mid‐epidermis, as well as prominent subcorneal pustules.…”

contrasting

confidence: 93%

IgA pemphigus associated with diffuse large B-cell lymphoma showing unique reactivity with desmocollins: unusual clinical and histopathological features

Asahina¹,

Koga²,

Suzuki³

et al. 2012

British Journal of Dermatology

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contrasting

confidence: 93%

IgA pemphigus associated with diffuse large B-cell lymphoma showing unique reactivity with desmocollins: unusual clinical and histopathological features

Asahina¹,

Koga²,

Suzuki³

et al. 2012

British Journal of Dermatology

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“…15,17,18 In our case, the detection of IgA autoantibodies not only to desmocollin 1 but also to the 2 other desmocollin isoforms may explain the finding of the staining throughout the epidermis by direct immunofluorescence microscopy. Kopp et al 19 recently described another case of SPD-type IgA pemphigus in which the autoantibodies reacted not only with the uppermost layers but throughout the entire epidermis. In this previous case, antibodies to both desmocollin 1 and desmoglein 1 were detected.…”

Section: Commentmentioning

confidence: 99%

Subcorneal Pustular Dermatosis–Type IgA Pemphigus With Autoantibodies to Desmocollins 1, 2, and 3

Düker¹,

Schaller²,

Rosé³

et al. 2009

Arch Dermatol

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Background: IgA pemphigus is a rare neutrophilic acantholytic autoimmune disease that is characterized by IgA deposits on keratinocyte cell surfaces. Clinically and histologically, IgA pemphigus is divided into 2 major subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. We report the first case of subcorneal pustular dermatosis-type IgA pemphigus that showed reactivity to all 3 isoforms of the desmocollin family by indirect immunofluorescence microscopy of COS7 cells transfected with desmocollin 1, 2, or 3. Observations: We describe a 94-year-old woman with IgA pemphigus with a unique immunopathologic pattern. Direct immunofluorescence microscopy revealed IgA deposits throughout the entire epidermis, with stronger staining in the upper epidermis. The autoantibodies from this patient did not show IgA or IgG reactivity with desmogleins via immunoblotting or enzyme-linked immunosorbent assay. By indirect immunofluorescence by the use of COS7 cells transfected with desmocollin 1, 2, or 3, IgA autoantibodies in a serum sample from our patient clearly reacted with all of them. Conclusions: The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially pemphigus and its subforms, are more complex than previously believed. Because pemphigus seems to be a heterogeneous disorder, further studies are needed to evaluate the complexity of the disease.

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“…Clinically, IgA pemphigus shows pustules, and histologically, both types identified show intraepidermal, subcorneal or spinous layer bullae, with hyperplastic epidermis, acantholytic keratinocytes, neutrophilic spongiosis, superficial inflammatory infiltrate with lymphocytes, neutrophils, and eosinophils, features which are also observed in other dermatoses ( Table III ) ( 11 , 17 , 19 ). However, the most accurate diagnostic test remains direct immunofluorescence ( 10 ).…”

Section: Spongiosis Patterns In Autoimmune Bullous Dermatosesmentioning

confidence: 82%

“…IgA pemphigus represents a rare entity of this group of bullous dermatoses (12). According to the site of bullae, there are two types of IgA pemphigus, namely III) (11,17,19). However, the most accurate diagnostic test remains direct immunofluorescence (10).…”

Section: Paraneoplastic Pemphigus (Pnp)mentioning

confidence: 99%

Spongiotic reaction patterns in autoimmune bullous dermatoses (Review)

et al. 2021

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Spongiosis or a spongiotic reaction pattern is the histological hallmark of intercellular epidermal edema, viewed as clear spaces within the epidermis. Although considered a histopathological term, spongiosis has clinical correlations, with the variable degrees of spongiotic reaction leading to different dermatological findings. This review aimed to highlight the spongiotic reactive patterns found in different autoimmune bullous dermatoses, considering the paucity of publications in this domain. The pathogenesis of spongiosis assumes the passage of extravasated edema fluid from the dermis into the epidermis, frequently accompanied by dermal inflammatory cells, and classification of the spongiotic reaction patterns, as well as their associated spongiotic dermatitis, take into consideration the type and distribution of these inflammatory cells. It is mandatory to consider different reactive processes, specific for other skin disorders, which act as simulants of different spongiotic patterns for the diagnosis. Considering the possible transient occurrence, the heterogeneity and non-specificity of the histopathological features of these diseases, the diagnosis is very complex, requiring clinicopathological correlations and additional analyses. A deep insight into spongiosis pathogeny may open the perspectives of a classification refinement of autoimmune bullous dermatoses.

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IgA pemphigus – Occurrence of anti‐Desmocollin 1 and anti‐Desmoglein 1 antibody reactivity in an individual patient

Abstract: Both desmocollin 1 and desmoglein 1 were autoantigens in this patient with IgA pemphigus and a distinct clinical presentation. To our knowledge, this is the first IgA pemphigus case with dual autoantibody reactivity.

Cited by 22 publications

References 19 publications

IgA pemphigus associated with diffuse large B-cell lymphoma showing unique reactivity with desmocollins: unusual clinical and histopathological features

IgA pemphigus associated with diffuse large B-cell lymphoma showing unique reactivity with desmocollins: unusual clinical and histopathological features

Subcorneal Pustular Dermatosis–Type IgA Pemphigus With Autoantibodies to Desmocollins 1, 2, and 3

Spongiotic reaction patterns in autoimmune bullous dermatoses (Review)

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