Paraneoplastic pemphigus occuring after radiotherapy for relapsed non-Hodgkin's lymphoma in a patient with common variable immunodeficiency.
Paraneoplastic pemphigus (PNP) is a rare multisystem, autoimmune disease with prominent mucocutaneous manifestations, occurring most commonly in association with haematological malignancies. It is characterised by the presence of circulating autoantibodies against epithelial adhesion proteins. We report a 46-year-old woman with common variable immunodeficiency who developed paraneoplastic pemphigus after receiving radiotherapy for relapsed non-Hodgkin's lymphoma. Flaccid bullae covering approximately 70% of the skin, painful oropharyngeal ulceration and periocular erosions were prominent clinical features. Despite supportive treatment and attempts at disease control using high-dose corticosteroids and cyclophosphamide, the patient became increasingly debilitated, developed septic shock secondary to Pseudomonas aeruginosa septicaemia on two occasions and died of respiratory failure 6 weeks after presentation. We highlight the need to be aware of (PNP) and to perform appropriate immunological investigations. In addition, we emphasise the importance of a multidisciplinary approach to the management of such patients.
Department of Haematology, Sunderland Royal Hospital, Sunderland, UK. g.l.stark@ncl.ac.il
G L
GL
Stark
D K
DK
Goff
G P
GP
Spickett
F
F
Wojnarowska
J A A
JA
Langtry
D017074:Common Variable Immunodeficiency / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000150:complications* / Q000175:diagnosis / Q000532:radiotherapy; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000209:etiology; D010392:Pemphigus / Q000209:etiology*; D018714:Radiotherapy, Adjuvant / Q000009:adverse effects*; D012131:Respiratory Insufficiency