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Pemphigus-like hypereosinophilic syndrome with FIP1L1-PDGFRA fusion gene: A challenging and uncommon clinical presentation.
- Source :
-
The Journal of dermatology [J Dermatol] 2019 Jun; Vol. 46 (6), pp. 531-534. Date of Electronic Publication: 2019 Apr 25. - Publication Year :
- 2019
-
Abstract
- Hypereosinophilic syndrome (HES) is often associated with cutaneous manifestations, mostly pruritic lesions, urticaria and angioedema. Mucosal lesions are rarely seen in HES but, when present, are usually the first manifestation of the disease. The clinical presentation may be heterogeneous, including erosions, aphthae or ulcers, and can be easily confused with other mucocutaneous disorders. Here, we present the case of a 64-year-old man with severe chronic erosive oral mucositis simulating pemphigus in which the finding of persistent eosinophilia and elevation of B <subscript>12</subscript> vitamin serum levels raised the suspicion of HES. The FIP1L1-PDGFRA fusion gene (4q12) was detected by fluorescence in situ hybridization and the patient was treated with imatinib mesylate with complete response of the disease.<br /> (© 2019 Japanese Dermatological Association.)
- Subjects :
- Diagnosis, Differential
Humans
Hypereosinophilic Syndrome drug therapy
Hypereosinophilic Syndrome genetics
Hypereosinophilic Syndrome immunology
Imatinib Mesylate pharmacology
In Situ Hybridization, Fluorescence
Male
Middle Aged
Mouth Mucosa immunology
Mouth Mucosa pathology
Oncogene Proteins, Fusion antagonists & inhibitors
Receptor, Platelet-Derived Growth Factor alpha antagonists & inhibitors
Severity of Illness Index
Stomatitis drug therapy
Stomatitis genetics
Stomatitis immunology
Treatment Outcome
mRNA Cleavage and Polyadenylation Factors antagonists & inhibitors
Hypereosinophilic Syndrome diagnosis
Imatinib Mesylate therapeutic use
Oncogene Proteins, Fusion genetics
Pemphigus diagnosis
Receptor, Platelet-Derived Growth Factor alpha genetics
Stomatitis diagnosis
mRNA Cleavage and Polyadenylation Factors genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1346-8138
- Volume :
- 46
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- The Journal of dermatology
- Publication Type :
- Academic Journal
- Accession number :
- 31021002
- Full Text :
- https://doi.org/10.1111/1346-8138.14888