title: Immunobullous dermatosis associated with Waldenström macroglobulinaemia treated with rituximab.
abstract: 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.
authors.affiliation: Department of Dermatology , Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
authors.forename: M
authors.initials: M
authors.lastname: Chattopadhyay
authors.forename: E
authors.initials: E
authors.lastname: Rytina
authors.forename: M
authors.initials: M
authors.lastname: Dada
authors.forename: B S
authors.initials: BS
authors.lastname: Bhogal
authors.forename: R
authors.initials: R
authors.lastname: Groves
authors.forename: S
authors.initials: S
authors.lastname: Handfield-Jones
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D000069283:Rituximab; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000150:complications*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007075:Immunoglobulin M; D000069283:Rituximab; D003520:Cyclophosphamide; D011239:Prednisolone