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Pemphigus‐like hypereosinophilic syndrome with FIP 1L1– PDGFRA fusion gene: A challenging and uncommon clinical presentation
Author(s) -
CurtoBarredo Laia,
Segura Sonia,
Ishii Norito,
Hashimoto Takashi,
Mascaró José M.,
Espinet Blanca,
Besses Carles,
Pujol Ramon M.
Publication year - 2019
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.14888
Subject(s) - medicine , hypereosinophilic syndrome , mucocutaneous zone , dermatology , eosinophilia , pemphigus , imatinib mesylate , pathology , disease , imatinib , immunology , myeloid leukemia
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 12 vitamin serum levels raised the suspicion of HES . The FIP 1L1– 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.