The expanding spectrum of IgA pemphigus: a case report and review of the literature
S. Geller
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorA. Gat
Department of Pathology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorT. Zeeli
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorA. Hafner
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorR. Eming
Department of Dermatology and Allergology, Philipps University, Biegenstrasse 10, 35037 Marburg, Germany
Search for more papers by this authorM. Hertl
Department of Dermatology and Allergology, Philipps University, Biegenstrasse 10, 35037 Marburg, Germany
Search for more papers by this authorCorresponding Author
E. Sprecher
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Correspondence
Eli Sprecher.
E-mail: [email protected]
Search for more papers by this authorS. Geller
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorA. Gat
Department of Pathology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorT. Zeeli
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorA. Hafner
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Search for more papers by this authorR. Eming
Department of Dermatology and Allergology, Philipps University, Biegenstrasse 10, 35037 Marburg, Germany
Search for more papers by this authorM. Hertl
Department of Dermatology and Allergology, Philipps University, Biegenstrasse 10, 35037 Marburg, Germany
Search for more papers by this authorCorresponding Author
E. Sprecher
Department of Dermatology, Tel Aviv Sourasky Medical Center, 6, Weizmann Street, Tel Aviv, 64239 Israel
Correspondence
Eli Sprecher.
E-mail: [email protected]
Search for more papers by this authorSummary
IgA 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 IgA dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD-type IGAP, we demonstrate the coexistence of IgA reactivity to both epidermal (desmocollins 2 and 3) and basement membrane-associated (BP180) proteins, suggesting the coexistence of atypical IGAP and linear IgA 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’.
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