Gressier Ludivine, Hotz Claire, Lelièvre Jean-Daniel, Carlotti Agnès, Buffet Marc, Wolkenstein Pierre, Bagot Martine, Melica Giovanna, Ortonne Nicolas
Department of Pathology, Henri Mondor Hospital, Public Assistance-Hospitals of Paris, France.
Arch Dermatol. 2010 Feb;146(2):165-9. doi: 10.1001/archdermatol.2009.359.
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).
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.
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.
华氏巨球蛋白血症的特异性皮肤病变罕见，包括肿瘤细胞浸润、IgM大疱性疾病以及所谓的IgM储存性丘疹，这些是皮肤巨球蛋白血症（CM）的特征。
我们报告了2例CM患者。在患者1中，CM起初表现为小丘疹，正如之前大多数已发表的CM病例研究中所报道的那样。在患者2中，病变进展严重，出现大的溃疡结节，且疾病进展迅速。如之前所描述患者中的半数情况一样，患者2怀疑有周围神经病变，患者1证实有周围神经病变，并产生了抗髓鞘相关糖蛋白抗体。
据我们所知，此前尚未有关于利妥昔单抗治疗与CM相关的华氏巨球蛋白血症的描述。利妥昔单抗使患者1的病变完全缓解，而患者2的疾病迅速进展并死亡。这些观察结果表明，皮肤IgM储存性病变的演变反映了潜在的华氏巨球蛋白血症的演变，且CM并非预后标志物。
