Disseminated cutaneous immunoglobulin M macroglobulinosis associated with cryoglobulinemia and minimal residual disease of Waldenström macroglobulinemia

Cutaneous macroglobulinosis with Waldenström macroglobulinemia

“… 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 Of these 9 cases, only one female case was reported, and this makes our case the second reported female case of CM in a patient with a history of WM. 3 Our case had peripheral neuropathy, which is also a rare association with CM. 1 The development of neuropathy is thought to be related to the accumulation of IgM in myelin sheaths.…”

“…Hence, CM can predict a latent plasma cell dyscrasia before any other clinical or pathologic evidence. 3 …”

“…Only 1 report showed weak positive staining for Congo red. 3 The most relevant diagnostic test is the detection of IgM by immunohistochemistry and/or direct immunofluorescence ( Table I ). Immunoelectron microscopy was found to clearly demonstrate the presence of large amounts of IgM in the dermis, which were found in the lesions of CM and in normal skin.…”

“…To our knowledge, only 9 cases of CM in patients with an established diagnosis of WM have been previously reported ( Table I ). 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 …”

“… The lesions were extremely painful to pressure. Soles of the feet 2290 mg/dL NM NM + - + by IHC NM NM Oshio-Yoshii et al 8 2017 63/M 1 Small reddish papules, some of which developed into discrete blister-like nodules On and around the right medial malleolus NM NM NM + - + by IHC Intravenous immunoglobulin therapy Ineffective Rituximab Clearance of the skin lesions leaving pigmented macules, but lesions recurred after 6 months Roupie et al 9 2019 65/M 3 Papules covered by a thick hyperkeratotic layer Soles of the feet NM NM NM + - + by IHC Rituximab, cyclophosphamide and corticosteroids Complete regression of skin lesions and a partial hematologic response Fayne et al 3 2019 56/F 0.5 Numerous crusted papules and nodules with eschars Disseminated across the body, including the face and fingers 280,200 g/dL NM NM + Weak + + by IHC R-CHOP, rituximab, bendamustine and ibrutinib Failed Current case 2020 50/F 0.5 Multiple erythematous to hyperpigmented papules and nodules with central crust Face, upper eyelids, and extensor aspects of both upper limbs 2200 mg/dL Generalized lymphadenopathy and HSM Peripheral Sensory neuropathy of both upper and lower limbs + - + by IHC R-CHOP Patient died after 2 sessions D...…”