Paraneoplastic pemphigus: a refractory autoimmune mucocutaneous disease.
OBJECTIVE
We report on a 42-year-old man with Stage IIA non-Hodgkin's lymphoma who developed a severe mucocutaneous blistering eruption. His diagnosis, paraneoplastic pemphigus, was based on clinical, histological, and immunofluorescence findings and confirmed by immunoprecipitation. Despite maximal therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil and the subsequent addition of cyclophosphamide and cyclosporine, the condition was fatal.


CONCLUSIONS
This case illustrates the refractory nature of this disease and the inadequacy of existing therapies.
Ng
Patricia P L
PP
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Rencic
Adrienne
A
Nousari
H Carlos
HC
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D017809:Fatal Outcome; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology*; D008297:Male; D009173:Mycophenolic Acid / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D010392:Pemphigus / Q000150:complications*; D010956:Plasmapheresis
D000305:Adrenal Cortex Hormones; D000903:Antibiotics, Antineoplastic; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide; D009173:Mycophenolic Acid