Conflict of interest: none declared.
Clinical dermatology ● Concise report
Immunobullous dermatosis associated with Waldenström macroglobulinaemia treated with rituximab
Article first published online: 18 JUN 2013
© 2013 British Association of Dermatologists
Clinical and Experimental Dermatology
Volume 38, Issue 8, pages 866–869, December 2013
How to Cite
Chattopadhyay, M., Rytina, E., Dada, M., Bhogal, B. S., Groves, R. and Handfield-Jones, S. (2013), Immunobullous dermatosis associated with Waldenström macroglobulinaemia treated with rituximab. Clinical and Experimental Dermatology, 38: 866–869. doi: 10.1111/ced.12166
- Issue published online: 20 NOV 2013
- Article first published online: 18 JUN 2013
- Manuscript Accepted: 21 JAN 2013
Waldenström macroglobulinaemia (WM) is a chronic lymphoproliferative disorder characterized by the presence of a monoclonal IgM paraprotein. Specific cutaneous features of WM include neoplastic cell infiltrates, IgM storage papules and IgM bullous dermatosis. We report a patient with subepidermal bullous disease associated with WM. Immunofluorescence identified IgM deposition along the basement membrane zone (BMZ) with circulating anti-BMZ IgM antibodies reacting with the dermal side of salt-split skin. The autoantibodies did not react with type VII collagen or laminin 332. Following failed treatment with doxycycline, prednisolone, intravenous immunoglobulin and dapsone, the patient was successfully treated with a modified RCVP regimen (rituximab, cyclophosphamide and prednisolone). To our knowledge, this is the first reported case of IgM bullous disease of WM treated with rituximab.