Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, benign, chronic disease characterized by sterile pustular eruption, typically involving the flexural sites of the trunk and proximal extremities. SPD is clinically and histopatologically identical to SPD-type IgA pemphigus. For differential diagnosis, immunologic evaluation is crucial. Here is presented an interesting case of differential diagnosis, in particular between SPD and SPD-type IgA pemphigus. Alternatively, SPD and SPD-type IgA pemphigus may be two extremes of a spectrum, with the same clinical and histologic presentation and a typical response to dapsone but with a different concentration of immunoglobulin at IFD and IFI, ranging from SPD (no IgA) to SPD-type IgA pemphigus (typical presence of IgA).

IgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases? / Aimo C.; Corra A.; Mariotti E.; Verdelli A.; Del Bianco E.; Bianchi B.; Maio V.; Salemme A.; Di Zenzo G.; Caproni M.. - In: ITALIAN JOURNAL OF DERMATOLOGY AND VENEREOLOGY. - ISSN 2784-8450. - ELETTRONICO. - 157:(2022), pp. 456-457. [10.23736/S2784-8671.22.07217-6]

IgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases?

Aimo C.;Mariotti E.;Verdelli A.;Del Bianco E.;Bianchi B.;Maio V.;Caproni M.
2022

Abstract

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, benign, chronic disease characterized by sterile pustular eruption, typically involving the flexural sites of the trunk and proximal extremities. SPD is clinically and histopatologically identical to SPD-type IgA pemphigus. For differential diagnosis, immunologic evaluation is crucial. Here is presented an interesting case of differential diagnosis, in particular between SPD and SPD-type IgA pemphigus. Alternatively, SPD and SPD-type IgA pemphigus may be two extremes of a spectrum, with the same clinical and histologic presentation and a typical response to dapsone but with a different concentration of immunoglobulin at IFD and IFI, ranging from SPD (no IgA) to SPD-type IgA pemphigus (typical presence of IgA).
2022
157
456
457
Aimo C.; Corra A.; Mariotti E.; Verdelli A.; Del Bianco E.; Bianchi B.; Maio V.; Salemme A.; Di Zenzo G.; Caproni M.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1309059
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