IgM-λ paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome
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.
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Available from: Roger Mouawad
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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.
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ABSTRACT: The aim of this review is to familiarize dermatologists and clinicians in general with cutaneous signs and symptoms that can help lead to an early diagnosis of an underlying malignancy. Because the skin is one of the most accessible organs, it should never be overlooked in systemic disease. Examination of the skin has the advantage of revealing important information about the patient's condition without requiring the use of invasive techniques. In the literature, most discussions of cutaneous manifestations of internal malignancy refer to classic paraneoplastic syndromes, but a wide variety of skin conditions, while not strictly paraneoplastic, can, in certain contexts, indicate the presence of malignancy or an increased risk of developing cancer later in life. In this review, various skin conditions that can signal malignancy or increased cancer risk are presented in randomly ordered groups based on clinical morphology. Conditions with multiple signs and symptoms have been classified on the basis of their most characteristic feature.
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