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.

journal_name

Clin Exp Dermatol

authors

Chattopadhyay M,Rytina E,Dada M,Bhogal BS,Groves R,Handfield-Jones S

doi

10.1111/ced.12166

subject

Has Abstract

pub_date

2013-12-01 00:00:00

pages

866-9

issue

8

eissn

0307-6938

issn

1365-2230

journal_volume

38

pub_type

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