Cutaneous macroglobulinosis: a case series

Abstract

Cutaneous macroglobulinosis is a rare skin manifestation of Waldenstrom macroglobulinemia. It is characterized by the deposition of eosinophilic, immunoglobulin‐derived material in the dermis. It typically presents as pink or skin‐colored papules favoring the extensor surfaces of the extremities. There are 11 reported cases of cutaneous macroglobulinosis in the literature. In our consultative dermatopathology practice we encountered three additional cases. In Case 1, a 41‐year‐old female with a monoclonal immunoglobulin M (IgM)‐kappa gammopathy developed skin‐colored papules on her extensor extremities, forehead and back. Histopathologic and immunohistochemical analyses revealed periodic acid‐Schiff (PAS)‐positive pink material in the dermis that stained with IgM. In Case 2, an 83‐year‐old female with a monoclonal IgM‐lambda paraproteinemia developed non‐blanching papules and plaques on the lower extremities and cheeks. Skin biopsies showed a striking occlusion of the vessels with a PAS‐positive eosinophilic precipitate, which was also found in the extravascular spaces and stained with IgM using direct immunofluorescence (DIF) and immunohistochemical stains. In Case 3, an 80‐year‐old male with Waldenstrom macroglobulinemia developed ulcerated papules and nodules on the lower extremities. The biopsy findings were similar to those of Case 2. We present a series of three patients with cutaneous macroglobulinosis and explore variations in the clinical and histopathological findings of this uncommon entity.

Cutaneous macroglobulinosis: successful treatment with rituximab

“…Regarding to RTX treatment against CM, there have been four reported cases: one case resulted in remission, another case remained unchanged and the other two cases got worse. 1,2,5 In the two cases got worse, RTX could not prevent the progression of the underlining WM. 1,2 In the case unchanged, a combination of bortezomib and RTX was introduced, and a partial response was reported with a reduction in M-spike but precise serum IgM levels were not shown.…”

“…The latter case can cause either cutaneous macroglobulinosis (CM) or so-called IgM bullous disease. 1,2 A 63-year-old Japanese man had been diagnosed with WM by an elevated IgM concentration with the presence of IgM-k monoclonal gammopathy in serum and an increase in lymphoplasmacytic cells in bone marrow for 1 year. He recognized small reddish papules on and around his right medial malleolar, and some of which developed into discrete blister-like nodules in a few weeks ( Fig.…”

“…Cutaneous macroglobulinosis, isolated storage of IgM in the dermis, is known to be a very rare condition and clinically presents translucent erythematous papules located on the limbs, buttocks and back. 2 Seventeen patients of CM have been reported in the English literature to date, 1-7 since the first case was reported as macroglobulinemia cutis in 1978. 3 Gressier et al summarized eight cases of CM and concluded that CM is not a prognostic marker, however, the intensity of CM may reflect circulating IgM level and disease severity.…”

Waldenström Macroglobulinemia: Clinical and Immunological Aspects, Natural History, Cell of Origin, and Emerging Mouse Models

“…Some symptoms of WM are attributable to tissue deposition of IgM, not to increased serum viscosity. IgM deposits may occur in kidney (glomeruli), intestine, and skin, leading to proteinuria, diarrhea, and characteristic papules (IgM storage papules or cutaneous macroglobulinosis) [12, 13], respectively. Kidney involvement usually leads to slowly progressive loss of function rather than acute renal failure [14, 15].…”

Plasmacytic cutaneous pathology: A review

“…Cutaneous macroglobulinosis results from deposition of IgM macroglobulins in the skin of a patient with Waldenstrom's macroglobulinemia or another IgM‐related paraproteinemia . It is characterized by flesh‐colored papules, mainly affecting acral sites.…”