Cutaneous macroglobulinosis: a report of 2 cases.
BACKGROUND
Specific cutaneous lesions of Waldenström macroglobulinemia are rare and include neoplastic cell infiltrates, IgM bullous disease, and so-called IgM-storage papules, which characterize cutaneous macroglobulinosis (CM).


METHODS
We report 2 patients with CM. In patient 1, CM started as small papules, as reported in most of the previously published case studies of CM. In patient 2, lesion evolution was remarkable by its severity, with large ulcerated nodules, and the disease progressed rapidly. As mentioned for half the previously described patients, peripheral neuropathy was suspected in patient 2 and demonstrated in patient 1, with production of antibodies to myelin-associated glycoprotein.


CONCLUSIONS
To the best of our knowledge, rituximab treatment of Waldenström macroglobulinemia associated with CM has not been described previously. Rituximab caused complete remission of the lesions in patient 1, whereas disease rapidly progressed in patient 2, and the patient died. These observations suggest that evolution of the cutaneous IgM-storage lesions reflects that of the underlying Waldenström macroglobulinemia, and CM is not a prognostic marker.
Department of Pathology, Henri Mondor Hospital, Public Assistance-Hospitals of Paris, France.
Ludivine
L
Gressier
Claire
C
Hotz
Jean-Daniel
JD
Lelièvre
Agnès
A
Carlotti
Marc
M
Buffet
Pierre
P
Wolkenstein
Martine
M
Bagot
Giovanna
G
Melica
Nicolas
N
Ortonne
D000368:Aged; D006801:Humans; D008297:Male; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D008258:Waldenstrom Macroglobulinemia / Q000150:complications / Q000473:pathology* / Q000628:therapy