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April 2009

Follicular Spicules of the Nose and Ears—Diagnosis

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Copyright 2009 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2009

Arch Dermatol. 2009;145(4):479-484. doi:10.1001/archdermatol.2009.33-b

Histopathologic examination of the skin biopsy specimen from the upper back area demonstrated follicular plugs of compact homogeneous eosinophilic material protruding from the epidermis, with no significant inflammatory infiltrate. Direct immunofluorescence staining detected IgG in the follicular plugs. A skin biopsy specimen from an area of necrotizing purpura revealed multiple thrombi of compact eosinophilic material in dilated capillaries in the upper dermis but no vasculitis, features that were consistent with thrombotic vasculopathy caused by cryoglobulinemia. Spicules from the nose were removed by gentle curretage and biochemically shown to be cryoprecipitates of monoclonal IgG-λ with electrophoretic characteristics identical to those of paraprotein and cryoprecipitates that were detected in serum and urine samples from the patient. A subsequent bone marrow biopsy revealed a hypercellular marrow with heavy infiltration of plasma cells (75%). These findings were consistent with the diagnosis of multiple myeloma with localized necrotizing livedo due to cryoglobulinemia and characteristic follicular spicules of the skin that were composed of the monoclonal protein found in the patient's serum sample. Oral dexamethasone and thalidomide therapy was initiated. However, after the rapid development of osteolyses and acute renal failure, the treatment was changed to bortezomib, doxorubicin hydrochloride, and dexamethasone. Within 10 weeks, the paraproteinemia had decreased substantially and the cutaneous spicules had completely resolved.

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