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The expanding spectrum of I g A pemphigus: a case report and review of the literature
Author(s) -
Geller S.,
Gat A.,
Zeeli T.,
Hafner A.,
Eming R.,
Hertl M.,
Sprecher E.
Publication year - 2014
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.12940
Subject(s) - pemphigus , autoantibody , medicine , dermatology , immunology , antibody
Summary I g A pemphigus ( IGAP ) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis ( SPD ) and intraepidermal neutrophilic I g A dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD ‐type IGAP , we demonstrate the coexistence of I g A reactivity to both epidermal (desmocollins 2 and 3) and basement membrane‐associated ( BP 180) proteins, suggesting the coexistence of atypical IGAP and linear I g A bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP , underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term ‘ IGAP spectrum’.