Case reportIgM-λ paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome
Section snippets
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,
References (30)
- et al.
POEMS syndrome: definitions and long-term outcome
Blood
(2003) - et al.
POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome
Am J Med
(1994) - et al.
Cutaneous angiomas in POEMS syndrome
J Am Acad Dermatol
(1985) - et al.
Multicentric angiofollicular lymph node hyperplasia associated with a solitary osteolytic costal IgG lambda myeloma: POEMS syndrome in a South American (Paraguayan) patient
Pathol Res Pract
(1989) - et al.
Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation
J Am Acad Dermatol
(1989) - et al.
Glomeruloid hemangioma leading to a diagnosis of POEMS syndrome
J Am Acad Dermatol
(2006) - et al.
Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the second international workshop on Waldenstrom's macroglobulinemia
Semin Oncol
(2003) - et al.
Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature
Blood
(2004) - et al.
The Crow-Fukase syndrome: a study of 102 cases in Japan
Neurology
(1984) Takatsuki syndrome: a reversible multisystem plasma cell dyscrasia
Arthritis Rheum
(1986)
Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature
Medicine (Baltimore)
Peripheral neuritis in myelomatosis
Br Med J
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome
Can J Neurol Sci
The skin changes of POEMS syndrome
Br J Dermatol
POEMS syndrome
Semin Dermatol
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