POEMS Syndrome

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Background

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystemic disease that occurs in the setting of a plasma cell dyscrasia.[1] The pathophysiologic link between the constellation of symptoms and the underlying disease is not well understood, but the link may be related to changes in the levels of a cytokine or a growth factor.

Described first by Crow in 1956 and then by Fukase in 1968, the syndrome was given the name Crow-Fukase syndrome (by which it is known in Japan) by Nakanishi in a study of 102 cases in Japan. In 1980, the acronym POEMS was coined by Bardwick et al on the basis of the aforementioned five main features of the disease.

Although no specific case definition has been established for POEMS syndrome, it has generally been agreed that patients with the syndrome should have three or more of the five main features.

In another approach to establishing the diagnosis, it was proposed that the presence of two major criteria (monoclonal plasma-proliferative disorder and polyneuropathy) along with at least one of seven minor criteria (sclerotic bone lesions, Castleman disease, organomegaly, edema, endocrinopathy, skin changes, and papilledema) is sufficient for diagnosis.[2] However, the findings of a retrospective analysis of 629 patients suggested that this approach may be inadequate for excluding other disease processes that may account for symptoms and that atypical presentations of POEMS may be misdiagnosed.[3, 4]

A refinement of this approach specified that three of five major criteria (the two mandatory ones and one of the three nonmandatory ones) plus at least one of six minor criteria are required.[5] The major criteria are as follows:

The minor criteria are as follows[5] :

The polyneuropathy associated with POEMS syndrome is bilateral and symmetric. It involves both motor and sensory nerves, begins distally, and has a progressive proximal spread. Associated cranial or autonomic nerves are not involved. Both demyelination and axonal degeneration are noted.[6]

The liver, the lymph nodes, and the spleen are the organs most frequently involved. Enlargement of the lymph nodes and spleen is secondary to changes consistent with Castleman disease (giant angiofollicular hyperplasia, multicentric plasma cell variant) in most patients. Approximately 15% of patients with POEMS syndrome have concomitant evidence of Castleman disease, and both may be associated with glomeruloid hemangioma.[7] Hepatomegaly is not associated with any defined histologic or pathophysiologic changes.

Multiple endocrinopathies have been associated with POEMS syndrome, and most patients have more than one endocrine abnormality. Many of the abnormalities noted can be explained by elevations in estrogen levels. Impotence and gynecomastia are common among men. Amenorrhea is common among women. Diabetes mellitus and glucose intolerance are also noted in many patients. Other associated endocrinopathies include hypothyroidism, hyperprolactinemia, and hypoparathyroidism.

POEMS syndrome is seen in the setting of a plasma cell dyscrasia. Although many plasma cell disorders have been reported in patients with this syndrome, most patients are seen with osteosclerotic myeloma or monoclonal gammopathy of unknown significance.

The M proteins most frequently found are the immunoglobulin A (IgA)-γ and immunoglobulin G (IgG)-γ light chains. In a case report of one patient with POEMS syndrome,[8] serum electrophoresis demonstrated an M-band with isolated IgA heavy chain but no abnormal light chain, which could suggest abnormal secretion of monoclonal protein or the rare possibility of coincidental heavy-chain disease in association with POEMS syndrome. A case of POEMS syndrome in association with Waldenström macroglobulinemia,[9] characterized by immunoglobulin M (IgM)-κ paraproteinemia, has been reported.

Classic multiple myeloma has not been associated with the disease. This plasma cell disorder has not been shown to be correlated with the constellation of symptoms noted in patients with POEMS syndrome.

POEMS syndrome has been associated with multiple dermatologic changes, the most common of which are hyperpigmentation, skin thickening, sclerodermoid changes, and hypertrichosis. Whitening of the proximal nail (Terry nails), peripheral edema, hyperhidrosis, clubbing of the fingers, Raynaud phenomenon, and angiomas have also been observed.

Additional signs and symptoms associated with POEMS syndrome include papilledema, anasarca, pleural effusions, ascites, fever, thrombosis, renal insufficiency, and diarrhea.

Pathophysiology

The pathophysiology of POEMS syndrome has not been well defined. It is known that in all patients, a plasma cell disorder underlies the development of the syndrome; however, the mechanism by which this occurs is not known. Elevations of cytokines, such as interleukin (IL)-1β, IL-6, and tumor necrosis factor (TNF)-α, have been noted.

In addition, significant elevations in vascular endothelial growth factor (VEGF) levels have been noted. Increased VEGF levels have been postulated to lead to enhanced vascular permeability, resulting in the associated edema, increased endoneural pressure, and deposition of plasma cell–derived material. As myelin is exposed to serum cytokines and complement, demyelination can occur.

In one case report of a patient with POEMS syndrome and bilateral cystoid macular edema, macular thickness varied with serum VEGF levels.[10] After vitrectomy and an intraocular triamcinolone injection, decreased macular thickness was associated with lower intraocular VEGF levels. The authors proposed that elevated VEGF levels may be causally related to cystoid macular edema in persons with POEMS syndrome. Stimulated vascular proliferation has also been postulated to result in some of the skin changes associated with the disease.

VEGF may also play a role in bone metabolism, as suggested by a report of two patients who received high-dose therapy (HDT) with autologous stem cell transplantation.[11] In a study by Kastritis et al,[12] decreasing VEGF levels corresponded with both clinical improvement and normalization of bone metabolism as measured by multiple remodeling indices.

In a study of 22 patients with POEMS, hyperalgesia was correlated with an elevation of proinflammatory cytokines (IL-1β, IL-6, and TNF-α), in addition to the electrophysiologic reduction of sensory nerve action potentials and the histopathologic loss of myelinated fibers.[13] Serum levels of epidermal growth factor (EGF), fibroblast growth factor (FGF), and platelet-derived growth factor (PDGF) have not been found to be increased in patients with POEMS syndrome.

The association of POEMS syndrome with Castleman disease and angioma formation led some to propose a role for human herpesvirus 8 (HHV-8); however, early studies did not confirm this role.

Etiology

All cases of POEMS syndrome are associated with a plasma cell disorder. The syndrome has been seen in association with osteosclerotic myeloma, monoclonal gammopathy of unknown significance, and Waldenström macroglobulinemia, but not with classic multiple myeloma. It is not clear why plasma cells in some dyscrasias produce factors that cause POEMS syndrome and those in others do not. The mechanism by which plasma cells lead to POEMS syndrome is not understood; however, elevations in IL-1β, IL-6, TNF-α, and VEGF levels have been implicated.

Epidemiology

POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized.

The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients.[14, 15]  POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

Prognosis

The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion, worst for those with a plasma cell disorder involving the bone marrow,[16] and intermediate for those with multiple lytic bone lesions. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. The morbidity associated with POEMS syndrome depends on the systems involved and can range from skin pigment alteration to debilitating weakness and loss of function.

The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). Miralles et al reported a 5-year survival rate of 60%.[17] Cardiorespiratory failure, renal failure, infection, and progressive inanition are among the most common causes of death. Overall shorter survival has been associated with extravascular volume overload (eg, effusions, edema, or ascites) and fingernail clubbing. Reduced survival times have been associated with the presence of cough, and in one study, respiratory weakness decreased the mean survival time from 139 months to 87 months.[18]

In a study (N = 49) of known deaths from POEMS syndrome at the Mayo Clinic during the period 2000-2022, Lee et al described a "capillary leak phenotype" (characterized by refractory ascites, effusions, and/or anasarca that ultimately resulted in hypotension, renal failure and cardiopulmonary arrest) that was found to be the cause of 19 of the 49 deaths (39%).[19]

Central and peripheral nervous system involvement can lead to significant morbidity and mortality.[20, 21, 22, 23]  The neurologic sequelae of POEMS syndrome cause approximately 50% of patients with POEMS syndrome to become bedridden. Death may also occur as a consequence of decubitus ulcers and thromboses due to inactivity, organomegaly, and endocrinopathy, rather than as a consequence of the aggressiveness of the monoclonal protein.

History

The presenting symptoms of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome vary according to the organ systems involved. Frequent presentations include the following:

Patients may also report the following:

Physical Examination

Skin manifestations of POEMS syndrome are as follows:

Neurologic manifestations are as follows:

Cardiovascular manifestations are as follows:

Endocrine manifestations are as follows:

Pulmonary manifestations are as follows:

Other extracutaneous manifestations are as follows:

Complications

In a review of 89 deaths occurring in POEMS syndrome patients treated at the Mayo Clinic between 2000 and 2022, 19 patients were found to have a syndrome of anasarca, effusions, and/or refractory ascites, which was considered a capillary leak phenotype (CLP) of POEMS syndrome.[19] Patients with this CLP phenotype died of hypotension, renal failure, and cardiopulmonary arrest.

Laboratory Studies

When clinical findings are suggestive of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is suggested clinically, a range of laboratory studies may be performed to define the extent of involvement and to establish whether other organ systems are involved.

Patients commonly have thrombocytosis with or without polycythemia.

Hypercalcemia and renal insufficiency are rarely present.

Generally, the M protein is immunoglobulin G (IgG)-γ or immunoglobulin A (IgA)-γ and small in size (median, 1.1 g/dL). Serum protein immunoelectrophoresis is used to define the nature and the extent of the monoclonal gammopathy.

Thyrotropin levels, fasting blood glucose levels, a glucose tolerance test, and estrogen levels can be used to screen for endocrinopathy.

Cerebrospinal fluid (CSF) may demonstrate a cytoalbuminologic dissociation. In patients with neuropathy, CSF test results either are in the reference range or may show elevated levels of protein.

Erythrocyte sedimentation rate (ESR) values are either in the reference range or slightly elevated.

For research purposes, cytokine and growth factor levels can be measured. Past studies have found that levels of tumor necrosis factor (TNF)-α, interleukin (IL)-6, IL-1β, and vascular endothelial growth factor (VEGF) are usually elevated, whereas levels of epidermal growth factor (EGF), fibroblast growth factor (FGF), and platelet-derived growth factor (PDGF) are within the reference range.

It has been suggested that POEMS syndrome may increase the risk of potentially lethal calciphylaxis, though this appears to be rare. Follow-up to watch for the occurrence of this symptom is advisable.[29]

Imaging Studies

Plain film radiographs are useful for locating lytic bone lesions caused by osteosclerotic myeloma.

At least 95% of patients have osteosclerotic lesions, with more than half having multiple lesions. Both osteosclerotic and osteolytic lesions may be present and may be of modest size.

Procedures

Because as many as 10% of patients have marrow involvement with plasma cells, bone marrow biopsy may be indicated.

Lymph node biopsy is indicated in patients with lymphadenopathy; in most patients, it demonstrates findings of Castleman disease. Human herpesvirus 8 (HHV-8) has been demonstrated within the lymphocytes of some of these lymph node biopsy specimens, in addition to being present within endothelial cells and lymphocytes in the glomeruloid hemangioma skin biopsy specimen.[30]  Within the HHV-8 genome, a viral homologue to human IL-6 is present, which is believed to induce angiogenesis and hematopoiesis. However, some patients test negative for HHV-8[31] ; thus, the complete role of this virus in the pathogenesis of Castleman disease remains to be defined.

Electromyography (EMG) yields findings consistent with polyneuropathy, prominent demyelination, and features of axonal degeneration. One study demonstrated a statistically significant pattern of lower limbs having absent or attenuated amplitudes of compound muscle action potentials and absent sensory nerve action potentials as compared with upper limbs. Intermediate nerve segments exhibited abnormal conduction slowing as compared with distal portions. These patterns may aid in early diagnosis.

Nerve biopsies usually reveal evidence of both axonal degeneration and demyelination, characterized by uncompacted myelin lamina without immunoglobulin deposition and minimal cellular infiltration.

Histologic Findings

The histopathologic changes seen in the sclerodermoid lesions are nonspecific, showing hyperpigmentation of the basal layer with an inflammatory infiltrate or dermal fibrosis. Other reports have noted vascular prominence. Sweat glands and collagen are normal, differentiating this condition from scleroderma. A skin biopsy of hyperpigmented lesional skin may demonstrate a nonspecific inflammatory infiltrate composed of a lymphoplasmacytic population.[32]

Most angiomas seen in persons with POEMS syndrome are histologically consistent with cherry angiomas. In a small proportion of patients, the angiomas have the appearance of a glomeruloid hemangioma.[33] This finding may be strongly suggestive of POEMS syndrome, but it is not pathognomonic, because the presence of this pathologic entity has been reported in a patient without POEMS syndrome. Multiple ectatic vascular spaces with luminal clusters of congested capillaries are noted in the lesions. The capillaries are surrounded by pericytes and resemble renal glomeruli (hence the term glomeruloid hemangioma).

A study of bone-marrow histology in 87 patients from the Mayo Clinic concluded that the constellation of lambda-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in bone marrow is highly suggestive of POEMS syndrome, especially in the context of a peripheral neuropathy.[34]

Medical Care

Treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome depends on  treatment of the underlying plasma cell disorder.[5] Most patients are treated with a combination of medical, surgical, and adjuvant therapies.[35]

The current mainstays of treatment for patients with diffuse disease include combinations of corticosteroids, low-dose alkylators, and peripheral blood stem cell transplantation following high-dose chemotherapy.[36, 37, 38] Some caution should be used in selecting the chemotherapeutic regimen to avoid worsening of the polyneuropathy.

Widespread osteosclerotic lesions may benefit from systemic therapy, with approximately one half of patients benefiting from melphalan and prednisone. Although about one quarter of patients respond to corticosteroids, relapses are common without additional treatment of the underlying plasma cell disorder. Intravenous immunoglobulin (IVIG) and plasmapheresis have not shown therapeutic benefit.

In a study from China, 31 patients received 12 cycles of oral melphalan (10 mg/m2 body surface area [BSA]) plus oral dexamethasone (40 mg/d) on days 1-4 of every 28 days.[39] Prophylaxis with a proton-pump inhibitor was added on days –1 to 5. Doses were adjusted according to side effects and renal function. Good hematologic and neurologic responses were reported.

For some patients, high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation may be considered. Preliminary studies reported a mortality of 7.4% associated with the procedure, in addition to significant peritransplantation morbidity that often necessitated intubation, but nearly all survivors experienced benefit. A case report described successful use of autologous hematopoietic stem cell transplantation to treat a patient with multicentric Castleman disease and POEMS syndrome, with subsequent improved nerve conduction and normalization of serum protein electrophoresis results.[40]

Treatment with high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation may result in clinical improvement of polyneuropathy.[10]

Relapse after successful autologous peripheral blood stem cell transplantation has been reported.[41]

A 2014 multicenter retrospective study in Japan showed positive results for autologous stem cell transplantation in the treatment of patients with POEMS syndrome in terms of long-term survival and quality of life.[42]

In a series of 30 POEMS syndrome patients treated at the Mayo Clinic in Rochester, MN, engraftment syndrome was reported in approximately 50% of patients, with symptoms including fever (93%), diarrhea (77%), weight gain (53%), and rash (43%), in addition to a 3% treatment-related mortality. Baseline splenomegaly was predictive of a complicated peritransplantation course.[43]

Kojima et al described clinical remission of more than 20 months for one patient who received high-dose chemotherapy with autologous CD34+-purged stem cells.[44]

The clinical course of one patient improved after administration of thalidomide, which has well-described antiangiogenetic, antiproliferative, and anticytokine characteristics.[45]

A case report of a patient with POEMS syndrome and elevated vascular endothelial growth factor (VEGF) levels described successful treatment with bevacizumab, an antiangiogenesis monoclonal antibody directed against VEGF. This treatment was associated with reduced edema and less painful neuropathy.[46]

Another patient treated with bevacizumab (10 mg/kg) twice monthly had decreased pain after 1 month, with improvements in breathing and walking within 10 weeks. These subjective changes were associated with a drop in VEGF levels and clinical improvements as measured by pulmonary function tests, electromyography (EMG), and nerve conduction studies. In this patient, neither hepatomegaly nor skin abnormalities were modified.[47]

Reported responses to bevacizumab have varied. In one patient treated 7 years after diagnosis, no clinical improvement was reported despite lowered VEGF levels, which the authors thought might be due to aberrant angiogenesis that had already developed systemically.[48]

Another POEMS patient treated with two courses of bevacizumab (5 mg/kg) had decreased VEGF levels but worsening paresis, eventually developing severe capillary leak syndrome with edema, ascites, and pleural effusion.[49] The authors encouraged caution before starting bevacizumab therapy because a sudden decrease in VEGF levels may result in apoptosis of endothelial cells, possibly increasing capillary leakiness. This approach is supported by another report of a patient treated with bevacizumab who developed diarrhea, generalized edema, pulmonary hypertension, and systemic inflammation and who subsequently died of multiorgan failure.

In a case report, one patient improved after receiving nine cycles of lenalidomide given daily for 21 days of a 28-day cycle with once-weekly dexamethasone.[50] The lenalidomide dose was gradually increased from 15 mg to the standard 25 mg. Lenalidomide is cytotoxic to malignant plasma cells in addition to being immunomodulatory. In 2009, a case report also described lenalidomide therapy in a patient with kappa restriction.[51]  

A 2015 prospective phase II trial of lenalidomide combined with dexamethasone (LEN-DEX) reported a promising reduction of VEGF to normal levels and recovery of neurologic function in many patients, with minimal adverse effects.[52]  A case report described significant hematologic and clinical improvements in a patient with POEMS syndrome after treatment with with a combination of daratumumab, lenalidomide, and dexamethasone.[53]

Sanada et al reported clinical remission and correspondingly decreased VEGF serum concentrations in one patient with POEMS syndrome and renal lesions after treatment with high-dose melphalan therapy followed by autologous blood stem cell transplantation.[54]

Authier et al reported a patient with POEMS syndrome associated with two monoclonal gammopathies (immunoglobulin G [IgG]-κ and immunoglobulin A [IgA]-γ) in whom high doses of all-trans-retinoic acid were potentially beneficial.[55]

Several reports have described successful therapy with bortezomib, used either alone or (as in more recent studies) in conjunction with dexamethasone.[56, 57, 58]

For patients with POEMS and pulmonary hypertension, high-dose steroid treatment appears to provide improvement, as confirmed by sequential hemodynamic studies.[59, 60]

Surgical Care

Surgical excision of isolated plasmacytomas may result in complete resolution of the syndrome.

Radiation treatment of solitary osteosclerotic lesions is first-line treatment for patients with an isolated plasmacytoma without bone marrow involvement.[1]

Radiation therapy in doses of 0.4-0.5 Gy to limited areas can improve osteosclerotic lesions in more than 50% of patients. More than 6 months may elapse before clinically apparent improvement is observed, and benefit may be observed even 2-3 years after therapy. Some authors have recommended systemic therapy for patients whose disease process fails to stabilize 3-6 months after the completion of radiation therapy.

Consultations

Because of the wide array of possible symptoms associated with POEMS syndrome, care of patients with this syndrome is coordinated through many specialists.

Once the diagnosis is clinically suggested, the patient should be evaluated by a neurologist, a hematologist, a dermatologist, and an endocrinologist to define the extent of disease. Referral to a pulmonologist or a nephrologist should be guided by the patient's symptoms. Consultation with a surgeon or a radiation oncologist may be needed for coordination of treatment.

Long-Term Monitoring

Even if the plasmacytoma shows a complete response to treatment, patients still need long-term follow-up care because some symptoms (eg, neurologic defects and functional loss due to tightening of the skin) may be permanent.

Prednisone (Deltasone, Orasone, Meticorten)

Clinical Context: 

Dexamethasone (Baycadron, Decadron DSC, Dexamethasone Intensol)

Clinical Context: 

Melphalan (Alkeran (DSC), Evomela, Hepzato)

Clinical Context: 

Cyclophosphamide (Cytoxan, Frindovxy)

Clinical Context: 

Bevacizumab (Alymsys, Avastin, Avzivi)

Clinical Context: 

Lenalidomide (Revlimid)

Clinical Context: 

Bortezomib (Boruzu, Velcade)

Clinical Context: 

Daratumumab (Darzalex)

Clinical Context: 

What is polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What are the signs and symptoms of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the pathophysiology of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What causes polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the prevalence of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What are racial predilections of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What are the sexual predilections of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which age groups are at highest risk for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the prognosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which history findings are characteristic of POEMS syndrome?Which physical findings are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which neurologic findings are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which cardiovascular findings are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which endocrine abnormalities are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which pulmonary findings are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What are the extracutaneous manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which conditions should be included in the differential diagnoses of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What are the differential diagnoses for POEMS Syndrome?What is the role of lab testing in the diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the role of imaging studies in the diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the role of biopsy in the diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which histopathologic findings are characteristic of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?How is polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome treated?What is the role of surgery in the treatment of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the role of radiation therapy in the treatment of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which specialist consultations are beneficial to patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is included in the long-term monitoring of patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?What is the role of drug treatment for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome?Which medications in the drug class Corticosteroids are used in the treatment of POEMS Syndrome?

Author

Joanna L Chan, MD, Mohs Fellow, California Skin Institute

Disclosure: Nothing to disclose.

Coauthor(s)

Matthew N Kubicki, MD, Resident Physician, University of Colorado Anschutz School of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Michael J Wells, MD, FAAD, Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Disclosure: Nothing to disclose.

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Neil Shear, MD, Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Disclosure: Nothing to disclose.

Wingfield Rehmus, MD, MPH, Dermatologist, BC Children's Hospital, Vancouver, British Columbia

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Valeant Canada<br/> Received honoraria from Valeant Canada for advisory board; Received honoraria from Pierre Fabre for advisory board; Received honoraria from Mustella for advisory board; Received honoraria from Abbvie for advisory board.

Acknowledgements

Alexa F Boer Kimball, MD, MPH Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital

Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

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