Case report
IgM-λ paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome

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POEMS is a rare multisystem paraneoplastic syndrome featuring polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein, and skin changes. In the relatively few reported biopsies of POEMS-associated cutaneous hyperpigmentation, the most common skin finding seen in patients with the disorder, only a non-specific inflammatory infiltrate has been demonstrated histologically. We present the case of a 79-year-old man with polyneuropathy, autoimmune thyroiditis, pancytopenia, and a history of lymphadenopathy who presented to the inpatient dermatology service with cutaneous hyperpigmentation. A skin biopsy of a hyperpigmented area showed a cutaneous lymphoplasmacytic infiltrate, prompting further investigation. A monoclonal IgM-λ paraprotein was subsequently identified, leading to administration of combination chemotherapy for a diagnosis of POEMS syndrome. The novel finding of a lymphoplasmacytic infiltrate in POEMS-associated hyperpigmentation suggests a diagnostic role for skin biopsy in these patients.

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Case report

A 79-year-old man with a 2-year history of progressive weakness, autoimmune thyroiditis, and recurrent edema, lymphadenopathy, and pancytopenia refractory to methylprednisolone, prednisone, intravenous immunoglobulin, and azathioprine, was evaluated for cutaneous hyperpigmentation. Physical examination revealed hyperpigmented macules and patches scattered over the trunk and proximal extremities (Fig 1). Additional findings included multiple well-demarcated brightly erythematous papules on the

Discussion

The diagnostic criteria for POEMS syndrome are controversial. The Mayo Clinic criteria for POEMS syndrome includes: (1) peripheral neuropathy; (2) monoclonal plasmaproliferative disorder; and (3) at least one of the following (excluding diabetes or thyroid abnormalities): sclerotic bone lesions, Castleman's disease, organomegaly (including splenomegaly, hepatomegaly, or lymphadenopathy), edema (including pleural effusions or ascites), endocrinopathy (adrenal, thyroid, pituitary, gonadal,

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    Funding sources: None.

    Conflicts of interest: None declared.

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