Keratotic vascular papules over the feet: A case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosis

King Edward Memorial Hospital, Mumbai, Maharashtra, India
Clinical and Experimental Dermatology (Impact Factor: 1.09). 10/2009; 35(3):278-81. DOI: 10.1111/j.1365-2230.2009.03651.x
Source: PubMed


Waldenström's macroglobulinaemia (WM) is a plasma-cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48-year-old man presented with a 4-year history of multiple painful, hyperkeratotic deep-seated papules over the pressure areas of both soles. He had a 1-year history of Raynaud's phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid-like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic-acid-Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone-marrow examination revealed a lymphoplasmacytoid malignancy. The patient's systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.

9 Reads
  • [Show abstract] [Hide abstract]
    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.
    Journal of Cutaneous Pathology 07/2012; 39(10):962-70. DOI:10.1111/j.1600-0560.2012.01983.x · 1.58 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Linear atrophoderma of Moulin is a rare, acquired, linear dermatosis. We present a 17-year-old girl with multiple asymptomatic brownish atrophic plaques in a zosteriform distribution on the left side of the trunk. Clinical presentation and dermatopathology was compatible with the diagnosis of linear atrophoderma. Twenty years after its initial description by Moulin, there are yet a limited number of case reports and unanswered questions regarding this entity.
    01/2013; 3(1):7-11. DOI:10.5826/dpc.0301a03

  • Journal of Cutaneous Pathology 05/2013; 40(5):442-4. DOI:10.1111/cup.12151 · 1.58 Impact Factor
Show more