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

ArticleinJournal of the American Academy of Dermatology 58(4):671-5 · May 2008with4 Reads
DOI: 10.1016/j.jaad.2007.07.021 · Source: PubMed
Abstract
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-lambda 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.
  • [Show abstract] [Hide abstract] ABSTRACT: Objective: To study the causes of misdiagnosis of patients with POEMS syndrome and to discuss the clues for its early diagnosis. Methods: The clinical and laboratory data of 26 inpatients with POEMS syndrome, who were treated in Changhai hospital over the last decade, were retrospectively analyzed. Results: The misdiagnosis rate of our group was 100%. The misdiagnosis was made in (3.31±0.97) hospitals and in (3.31±0.93) clinical departments; the misdiagnosis period was (19.42±10.86) months and it had been misdiagnosed as 18 other diseases. The initial symptoms included polyneuropathy in 21 (80.8%) cases, edema of lower extremity in 22 (84.6%) cases, and body weight loss in 8 (30.8%) cases. The typical clinical symptoms included polyneuropathy in 26 (100%) cases and organomegaly in 24 (92.3%). Two cases had newly identified uterine hypertrophy, one had adrenal gland hypertrophy, and one had gastric wall thickening mimicking advanced gastric cancer. Hypothyroidism, impotence, skin pigmentation and sclerosis occurred in 76.9% (20/26), 60% (6/10), 92.3% (24/26) and 65.4% (17/26) cases, respectively. Monoclonal plasma cell proliferation was documented in 18 (81.8%); M proteins were positive in 14 (63.6%) cases by serum immunofixation, and only 2 (9.1%) cases also positive by serum protein electrophoresis. One patient was positive of M protein by urine immunofixation and one had abnormal infiltration of plasma cells in the gastric wall. Lymph node biopsy were performed in 8 patients and 3 were found to have Castleman disease; the other 5 cases had lymphocyte infiltration, with 3 complicated with plasma cell proliferation. Nerve biopsy in 4 cases all revealed axonal degeneration and one patient complicated with demyelination. Bone marrow biopsy in 5 cases revealed plasmacytosis in 2 cases and myeloma in one. Excessive radioactivity resorption was found in 10 of the 16 cases (62.5%) and abnormal plasma cells were detected in 2 cases by bone aspiration guided by radioisotope hone scan. Conclusion: Misdiagnosis of POEMS syndrome is very common. Polyneuropathy, edema of lower extremity and body weight loss are the common early symptoms of POEMS syndrome. Early diagnosis can be made by having an intimate knowledge of the progression of the disease and by detecting M protein through various approaches.
    Article · Jun 2008
  • Article · Oct 2008
  • [Show abstract] [Hide abstract] ABSTRACT: To investigate skin manifestations of the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and their correlation with serum vascular endothelial growth factor (s-VEGF-A) levels and to describe the impact of autologous peripheral blood stem cell transplantation (aPBSCT) on these manifestations and the correlation with s-VEGF-A levels. Case series from January 1993 through June 2007. Hospitalized care in Assistance Publique-Hôpitaux de Paris in Pitié-Salpêtrière and Tenon hospitals. Twenty-three patients with POEMS syndrome, 10 of whom were clinically followed up after aPBSCT. Description and distribution of clinical lesions at POEMS syndrome diagnosis, skin evaluation after aPBSCT, and s-VEGF-A levels measured at POEMS syndrome diagnosis and after aPBSCT. In 21 patients with skin manifestations at POEMS syndrome diagnosis, the most common skin manifestations were hemangiomas (18 patients [86%]), hyperpigmentation (16 [76%]), skin thickening (12 [57%]), acrocyanosis (12 [57%]), hypertrichosis (11 [52%]), acquired facial lipoatrophy (11 [52%]), and white nails (8 [38%]). The median s-VEGF-A level was not different between patients with and without skin manifestations except in those with hypertrichosis (P = .04). After aPBSCT, no significant correlation was observed between s-VEGF-A level decreases and response of skin manifestations, again except for hypertrichosis (P = .007). Acquired facial lipoatrophy and livedo should be added to the skin manifestations of POEMS syndrome. Despite a role of s-VEGF-A in various skin manifestations, the impact of s-VEGF-A level decreases on skin outcomes is weak after aPBSCT, mostly resulting in clinical stabilization.
    Full-text · Article · Jun 2010
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