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Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature
Author(s) -
Barnadas MA,
Roe E,
Brunet S,
Garcia P,
Bergua P,
Pimentel L,
Puig L,
Francia A,
García R,
Gelpí C,
Sierra J,
Coll P,
Alomar A
Publication year - 2006
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1111/j.1468-3083.2005.01345.x
Subject(s) - medicine , rituximab , paraneoplastic pemphigus , pemphigus , dermatology , methylprednisolone , lymphoma , disease , mucocutaneous zone , cd20 , immunology , autoantibody , antibody
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease with poor prognosis when associated with malignant neoplasm. We report the case of a patient with PNP associated with a CD20+ non‐Hodgkin follicular lymphoma who was treated with Rituximab plus corticosteroids and short courses of cyclosporin. One and a half years after Rituximab therapy, oral ulcerations had cleared and oral methylprednisolone was slowly tapered down without further recurrences. In the course of the disease, the patient developed sepsis due to Listeria monocytogenes and viral infections by human herpes virus 1 and 3. At the end‐stage of the disease she developed a cutaneous infection from Mycobacterium chelonae . The patient died 2 years and 7 months after the onset of PNP. Rituximab may be useful for PNP therapy, but further studies are necessary to confirm this hypothesis.