Paraneoplastic
pemphigus (PNP) is a rare autoimmune bullous disease associated with
neoplasm, which is clinically characterized by mucocutaneous lesions resembling
pemphigus vulgaris or
erythema multiforme. A case presented with PNP refractory to
chemotherapy including
rituximab, predonisolone and
cyclophosphamide (RCHOP regimen). A 36-year-old man, who had been diagnosed as extended
follicular lymphoma, presented with a polymorphous skin eruption of the trunk, sclera
conjunctivitis, and severe mucosal erosions of the lips and oral cavity. He was diagnosed as
pemphigus pathologically by a biopsy of the oral mucosa. However, 3 courses of
rituximab and CHOP
therapy, which exert a partial response with
lymphoma lesions, did not prove effective for oral
stomatitis due to
pemphigus. He received
corticosteroid therapy (
prednisolone 40 mg/day) and went into a state of temporally remission regarding
pemphigus. However, the mucosal lesions were again exacerbated despite control of the
lymphoma status after
chemotherapy. Oral
stomatitis extended to the upper respiratory system through the larynx and resulted in
bronchiolitis obliterance clinically presented likely as severe
chronic obstructive pulmonary disease (
COPD). Because it is known that PNP refractory to long-term
steroid and cytoreductive
therapy has a progressive character and poor prognosis, supportive care would be warranted for these patients.