Kennedy Disease Misdiagnosed as Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Case Report
نویسندگان
چکیده
OBJECTIVE The aim of this paper was to report the first case of Kennedy disease misdiagnosed as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. CLINICAL PRESENTATION AND INTERVENTION A 58-year-old Chinese man presented with limb numbness, progressive limb proximal weakness, lymph node and thyroid enlargement, edema, pigmentation in the lower limb, and obvious gynecomastia, which was initially diagnosed as POEMS syndrome and was treated with dexamethasone and small doses of cyclophosphamide without any improvement after 6 months. Finally, the patient diagnosis was confirmed as Kennedy disease (KD) by gene analysis. CONCLUSION This case suggests that clinicians should pay more attention to the differential diagnosis between KD and POEMS syndrome. Gene analysis was helpful in detecting this rare confusing disease in this patient.
منابع مشابه
Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome
POEMS syndrome (Crow-Fukase syndrome) is a rare paraneoplastic disorder. It is characterized by peripheral neuropathy, elevated vascular endothelial growth factors (VEGFs), monoclonal gammopathy, sclerotic bone lesions and Castleman disease. Other important clinical features are organomegaly, edema, ascites, papilledema, endocrinopathy, skin changes and thrombocytosis. A high index of suspicion...
متن کاملگزارش یک مورد نشانگان پوئمز (POEMS SYNDROME) از ایران و مروری بر مقالاتی که در این زمینه منتشر شده است.
The crow-fukas, Takatsuki or POEMS syndrome (The acronym of Polyneuropathy, Organomegaly, endocrinopathy, M component and skin changes), a rare, multisystem disorder associated with osteosclerotic myeloma, is characterize by the combination of plasma cell discrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal(M) protein, skin changes, as well as various other sign, such...
متن کاملAn Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS)
POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS...
متن کاملThe first case of POEMS syndrome with synchronous breast cancer: What are the associated diagnostic challenges?
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes (POEMS) syndrome is a rare plasma cell disorder that causes a paraneoplastic syndrome. We report the first case of POEMS syndrome with synchronous breast cancer. The patient was at risk of being misdiagnosed with metastatic cancer, and it is important to emphasize that physical examinations provided vital diagnost...
متن کاملPoems Syndrome in A-10-year-old Gi̇rl: a Case Report Poems Sendromu: 10 Yaşinda Kiz Olgu
POEMS syndrome has been defined as an association of plasma celi dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. In this report, we describe a case of POEMS syndrome. A 10-year-old girl presented with polyneoropathy, diabetes mellitus type I, double monoclonal gammopathy (IgA and IgG), hepatomegaly, cardiomegaly, hyperpigmentation, and hypertricosis. To ...
متن کامل