The expanding spectrum of<scp>I</scp>g<scp>A</scp>pemphigus: a case report and review of the literature

Geller, Shamir; Gat, Andrea; Zeeli, Tal; Häfner, A.; Eming, Ruediger; Hertl, Michael; Sprecher, Eli

doi:10.1111/bjd.12940

Cited by 31 publications

(37 citation statements)

References 35 publications

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“…SPD and SPD-type IgA pemphigus are indistinguishable clinically and histologically [56]. IgA pemphigus is an intraepidermal blistering disease with circulating and tissue-bound IgA autoantibodies against keratinocyte cell surfaces [56].…”

Section: Differential Diagnosesmentioning

confidence: 99%

“…IgA pemphigus is an intraepidermal blistering disease with circulating and tissue-bound IgA autoantibodies against keratinocyte cell surfaces [56]. Circulating IgA is present in 50 % of cases [13].…”

Section: Differential Diagnosesmentioning

confidence: 99%

“…The pustules then coalesce into an annular, circinate pattern with crusts centrally. Pruritus is a feature in about 50 % of cases [56,57], and mucosal involvement is usually spared [56,59]. Histology reveals neutrophilic infiltration in the epidermis, clefts and pustules in the subcorneal region, and mild or no acantholysis [11,13,56,57].…”

Section: Differential Diagnosesmentioning

confidence: 99%

“…Histology reveals neutrophilic infiltration in the epidermis, clefts and pustules in the subcorneal region, and mild or no acantholysis [11,13,56,57]. Since SPD-type IgA pemphigus is indistinguishable from SPD clinically and histologically [56], immunologic evaluation is crucial. Several methods are available to detect the presence or absence of tissuebound IgA deposits.…”

Section: Differential Diagnosesmentioning

confidence: 99%

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Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress

2016

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Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, benign yet relapsing pustular dermatosis. Its incidence and prevalence have not been well studied. It characteristically presents as hypopyon pustules on the trunk and intertriginous areas of the body. SPD is similar to two other disease entities. Both SPD-type immunoglobulin (Ig)-A pemphigus and annular pustular psoriasis clinically and histologically present similarly to SPD. Immunologic studies separate SPD-type IgA pemphigus from SPD and pustular psoriasis. However, there is still an unclear designation as to whether SPD is its own entity distinct from pustular psoriasis, as the once thought characteristic histologic picture of psoriasis does not hold true for pustular psoriasis. SPD has been reported to occur in association with several neoplastic, immunologic, and inflammatory conditions. Dapsone remains the first-line treatment for SPD, although dapsone-resistant cases have been increasingly reported. Other therapies have been used singly or as adjunctive therapy with success, such as corticosteroids, immunosuppressive agents, tumor necrosis factor inhibitors, and ultraviolet light therapy. This article provides a review of the last 30 years of available literature, with a focus on successful treatment options and a suggestion for reappraisal of the classification of SPD.

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Section: Differential Diagnosesmentioning

confidence: 99%

Section: Differential Diagnosesmentioning

confidence: 99%

Section: Differential Diagnosesmentioning

confidence: 99%

Section: Differential Diagnosesmentioning

confidence: 99%

See 2 more Smart Citations

Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress

2016

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“…Binding of IgA antibodies to keratinocyte cell surface antigens leads to accumulation of neutrophils in the epidermis, resulting in intraepidermal blistering (2). Depending on blister location, two variants of the disease have been recognized: 1) subcorneal pustular dermatosis (SPD) and 2) intraepidermal neutrophilic (IEN) IgA dermatosis (3).…”

mentioning

confidence: 99%

IgA Pemphigus in a Child – a Case Report

Lekić

Gajić-Veljić

Popadić

et al. 2017

Serbian Journal of Dermatology and Venereology

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IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

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