View source for POEMS syndrome

{{redirect|POEMS|other uses|Poetry (disambiguation)}}
{{short description|Paraneoplastic syndrome}}
{{Infobox medical condition (new)
| name             = POEMS syndrome
|synonyms          = Crow–Fukase syndrome, Osteosclerotic myeloma, PEP syndrome, Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome, Takatsuki syndrome.<ref name=rarediseases>{{cite web|access-date=2023-08-14|title=POEMS syndrome&nbsp;— About the Disease&nbsp;— Genetic and Rare Diseases Information Center|url=https://rarediseases.info.nih.gov/diseases/7411/poems-syndrome|website=rarediseases.info.nih.gov}}</ref>
| image            = [[File:Plasma cells.jpg|Plasma cells|250px]]
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| alt              = [[Micrograph]] of [[plasma cells]].
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| caption          = [[Multiple myeloma]] is usually diagnosed because [[malignant]] [[plasma cells]] continue to produce an antibody that can be detected as a [[paraprotein]].
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|symptoms          = [[Papilledema]],<ref name="pmid21035860"/> [[polyneuropathy]],<ref name=rarediseases/> [[edema]],<ref name="pmid28299525"/><ref name=ajh/> [[hyperpigmentation]], [[hypogonadism]],<ref name=rarediseases/> [[Organomegaly|visceromegaly]],<ref name=rarediseases/> [[amenorrhea]],<ref name="pmid28299525"/><ref name=ajh/> [[gynecomastia]],<ref name="pmid28299525"/><ref name=ajh/> [[erectile dysfunction]],<ref name="pmid28299525"/><ref name=ajh/> and [[testicular atrophy]].<ref name="pmid28299525"/><ref name=ajh/>
|complications     = [[Multiple organ dysfunction syndrome|Multiorgan failure]], [[restrictive lung disease]], [[pulmonary hypertension]], [[Respiratory arrest|cardiorespiratory failure]], [[Starvation|progressive inanition]], [[infection]], [[capillary leak syndrome|capillary leak-like syndrome]], and [[Kidney failure|renal failure]].<ref name=mjhid>{{cite journal|first1=Andrea|last1=Nozza|title=Poems Syndrome: An Update |url=https://www.mjhid.org/|journal=Mediterranean Journal of Hematology and Infectious Diseases|date=1 September 2017|issn=2035-3006|pages=e2017051|volume=9|issue=1|doi=10.4084/mjhid.2017.051|pmid=28894560 |pmc=5584767 }}</ref>
|onset             = 5th to 6th decade of life.<ref name=medscape>{{cite web|title=POEMS Syndrome: Background, Pathophysiology, Etiology|url=https://emedicine.medscape.com/article/1097031-overview|date=16 October 2021}}</ref>
|duration          = Chronic.<ref name=medscape/>
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|diagnosis         = Clinical Criteria.<ref name=medscape/>
|differential      = [[Chronic inflammatory demyelinating polyneuropathy|Chronic inflammatory demyelinating polyradiculoneuropathy]], [[cryoglobulinemia]], [[amyloidosis|nodular localized cutaneous amyloidosis]], [[multiple myeloma]], [[monoclonal gammopathy of undetermined significance]], [[Waldenström macroglobulinemia|waldenstrom hypergammaglobulinemia]], [[scleroderma]], [[raynaud phenomenon]], [[AL amyloidosis|amyloid light chains (AL) amyloidosis]], [[Guillain–Barré syndrome|guillain-Barré syndrome]].<ref name=medscape/><ref name=nord>{{cite web|access-date=2023-08-16|title=POEMS Syndrome&nbsp;— Symptoms, Causes, Treatment&nbsp;— NORD|url=https://rarediseases.org/rare-diseases/poems-syndrome/|website=rarediseases.org}}</ref>
| prevention       =
|treatment         = [[Corticosteroid]]s, low-dose alkylators, peripheral [[Hematopoietic stem cell transplantation|blood stem cell transplantation]], high-dose [[chemotherapy]].<ref name=medscape/>
|medication        = [[Thalidomide]], [[lenalidomide]], [[bortezomib]], [[bevacizumab]].<ref name=mjhid/>
|prognosis         = Median survival of 13.8 years.<ref name=ashpub>{{cite journal|last1= Dispenzieri|first1= Angela|date= 1 January 2005|title= POEMS Syndrome|journal= Hematology Am Soc Hematol Educ Program|volume= 2005|issue= 1|pages= 360–357|publisher= ash publications|doi= 10.1182/asheducation-2005.1.360|pmid= 16304404|issn= 1520-4391|url=https://ashpublications.org/hematology/article/2005/1/360/19304/POEMS-Syndrome|access-date= 15 August 2023|doi-access= free}}</ref>
| frequency        = Rare
| deaths           =
| named after      = <!-- or
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}}
'''POEMS syndrome''' (also termed '''osteosclerotic myeloma''', '''Crow–Fukase syndrome, Takatsuki disease''', or '''PEP syndrome''') is a [[rare disease|rare]] [[paraneoplastic]] [[syndrome]] caused by a [[clone (cell biology)|clone]] of aberrant [[plasma cell]]s. The name POEMS is an [[acronym]] for some of the disease's major [[medical sign|signs]] and [[symptom]]s ([[polyneuropathy|'''p'''olyneuropathy]], [[organomegaly|'''o'''rganomegaly]], [[endocrinopathy|'''e'''ndocrinopathy]], [[myeloma protein|'''m'''yeloma protein]], and [[cutaneous condition|'''s'''kin changes]]), as is PEP ([[polyneuropathy|'''p'''olyneuropathy]], [[endocrinopathy|'''e'''ndocrinopathy]], [[plasma cell dyscrasia|'''p'''lasma cell dyscrasia]]).

The signs and symptoms of most [[neoplasms]] (excessive, abnormal tissue growths) are due to their [[Mass effect (medicine)|mass effects]] (compression of surrounding tissue by the mass of the growth) caused by the invasion and destruction of tissues by the neoplasms' cells. Signs and symptoms of a cancer causing a [[paraneoplastic syndrome]] result from the release of [[humoral]] factors such as [[hormone]]s, [[cytokine]]s, or [[immunoglobulins]] by the syndrome's neoplastic cells and/or the response of the [[immune system]] to the neoplasm. Many of the signs and symptoms in POEMS syndrome are due at least in part to the release of an aberrant [[Antibody|immunoglobulin]], i.e. a [[myeloma protein]], as well as certain [[cytokine]]s by the [[Malignancy|malignant]] [[plasma cell]]s.<ref name="pmid27866585">{{cite journal | vauthors = Castillo JJ | title = Plasma Cell Disorders | journal = Primary Care | volume = 43 | issue = 4 | pages = 677–691 | year = 2016 | pmid = 27866585 | doi = 10.1016/j.pop.2016.07.002 }}</ref><ref name="pmid28299525">{{cite journal | vauthors = Warsame R, Yanamandra U, Kapoor P | title = POEMS Syndrome: an Enigma | journal = Current Hematologic Malignancy Reports | volume = 12 | issue = 2 | pages = 85–95 | year = 2017 | pmid = 28299525 | doi = 10.1007/s11899-017-0367-0 | s2cid = 31324035 }}</ref><ref name=ajh/>

POEMS syndrome typically begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women.

== Signs and symptoms ==
The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 13–18 months) between the onset of initial symptoms and diagnosis.<ref name="pmid27866585"/><ref name="pmid28299525"/> In addition to the signs and symptoms indicated by the POEMS acronym, the PEST acronym is used to describe some of the other signs and symptoms of the disease. PEST stands for [[papilledema|'''P'''apilledema]], evidence of '''E'''xtravascular volume overload ([[ascites]], [[pleural effusion]], [[pericardial effusion]], and lower extremity [[edema]]), [[Bone metastasis#Diagnosis|'''S'''clerotic bone lesions]], and [[thrombocytosis|'''T'''hrombocytosis]]/[[erythrocytosis]] (i.e. increased in blood [[platelets]] and [[erythrocytes|red blood cells]]). Other features of the disease include a tendency toward [[leukocytosis]], [[Thrombosis|blood clot formation]], abnormal lung function ([[restrictive lung disease]], [[pulmonary hypertension]], and [[Diffusion capacity|impaired lung diffusion capacity]]), very high blood levels of the [[cytokine]] [[vascular endothelial growth factor|vascular endothelial growth factor (VEGF)]], and an overlap with the signs and symptoms of multicentric [[Castleman disease]].<ref name="pmid28299525"/>

=== Common features ===
The more common features of the disease are summarized in the acronym POEMS:
'''[[Papilledema]]''' (swelling of the [[optic disc]]) often but not always due to increased [[intracranial pressure]]) is the most common ocular sign of POEMS syndrome, occurring in ≥29% of cases. Less frequent ocular findings include [[cystoid macular edema]], [[Retinal detachment#Types|serous macular detachment]], [[Idiopathic orbital inflammatory disease|infiltrative orbitopathy]], and venous sinus thrombosis.<ref name="pmid21035860">{{cite journal | vauthors = Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A | title = Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome | journal = Ophthalmology | volume = 118 | issue = 4 | pages = 778–82 | year = 2011 | pmid = 21035860 | doi = 10.1016/j.ophtha.2010.08.013 }}</ref> Some features have been observed in patients with POEMS syndrome but are not yet certain to form part of the syndrome itself. These include [[thrombophilia|a predisposition to forming blood clots]], [[arthralgia|joint pain]], [[cardiomyopathy]] (systolic dysfunction), [[fever]], low [[vitamin B12]] levels, and [[diarrhea]].<ref name=Dispenzieri/>

== Pathogenesis ==
While the main features of this [[Paraneoplastic syndrome|paraneoplastic disease]] have been described, the exact mechanism behind its development, progression, and manifestations remain elusive. Overproduction of the [[myeloma protein]] and [[Vascular endothelial growth factor|VEGF]] may underlie some, but are insufficient to explain all, of the multi-organ features of the disease. It is suggested that various other [[cytokine]]s produced by the clonal plasma cells, perhaps working in concert with each other as well as with [[Vascular endothelial growth factor|VEGF]] and the [[myeloma proteins]], mediate many of the features of POEMS syndrome. The other [[cytokine]]s detected in, and suspected of contributing to, POEMS syndrome include [[IL1B|interleukin 1β]], [[interleukin 6]], and [[TNFα]]. Nonetheless, it seems likely that some of these paraneoplastic factors, operating individually, make a major contribution to certain features of the disease. For example, [[Vascular endothelial growth factor|VEGF]], given its ability to stimulate [[angiogenesis|blood vessel formation]], would seem likely to be the major contributor to the pathologic hyper-vascularization changes seem in many tissues, such as [[lymph node]]s, affected by POEMS syndrome.<ref name=Dispenzieri>{{cite journal |vauthors=Dispenzieri A, Kyle RA, Lacy MQ, etal |title=POEMS syndrome: definitions and long-term outcome |journal=Blood |volume=101 |issue=7 |pages=2496–506 |year=2003 |pmid=12456500 |doi=10.1182/blood-2002-07-2299 |doi-access=free }}</ref>

== Diagnosis ==
The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria.<ref name=ajh/>

=== Mandatory criteria ===
==== Polyneuropathy ====
{{main|Polyneuropathy}}
[[Subacute]], distal, symmetrical [[Sensorimotor polyneuropathy|sensorimotor neuropathy]] with [[allodynia]] and [[hyperpathia]] is the most frequent presentation of POEMS syndrome. [[Peripheral neuropathy|Neuropathy]] is often the first trait, and it may be the only initial symptom. Clinical examination may show [[Wasting|distal wasting]], [[weakness]], and sensory impairment affecting both large and small [[Stimulus modality|fibre sensory modalities]].<ref name=clinicalupdate>{{cite journal|last1= Brown|first1= Rachel|last2= Ginsberg|first2= Lionel|date= 1 January 2019|title= POEMS syndrome: clinical update|journal= Journal of Neurology|volume= 266|issue= 1|pages= 268–277|doi= 10.1007/s00415-018-9110-6|pmid= 30498913|pmc= 6342878}}</ref>

==== Plasma cell dyscrasia ====
{{main|Plasma cell dyscrasias}}
[[Plasma cell dyscrasias]] are a group of [[Monoclonal gammopathy|monoclonal gammopathies]] in which normal [[plasma cell]]s in the [[bone marrow]] and [[soft tissue]]s become altered. POEMS syndrome is often associated with an [[Immunoglobulin A|IgA]] or [[Immunoglobulin G|IgG]] lambda limited [[plasma cell]] dysfunction. On [[iliac crest]] [[Biopsy|biopsies]], patients with POEMS syndrome often have few [[Monoclonal antibody|monoclonal plasma cells]]. In patients with localized illness, [[iliac crest]] [[Biopsy|biopsies]] may be normal. Other common findings were [[megakaryocyte]] [[hyperplasia]] and clustering, as well as unusual [[megakaryocyte]] appearances. Atypical [[plasma cell]]s invade normal marrow in [[Osteosclerosis|osteosclerotic]] [[lesion]]s, causing [[Sclerosis (medicine)|sclerosis]] of the [[Lamella (cell biology)|bony lamellae]].<ref name=clinicalupdate/>

=== Major criteria ===
==== Castleman's disease ====
{{main|Castleman disease}}
[[Castleman disease|Castleman's disease]] (CD) is a rare [[Homogeneity and heterogeneity|heterogeneous]] [[lymph node]] disorder characterized by elevated [[Interleukin 6|interleukin-6]] levels. [[Peripheral neuropathy|Neuropathy]] can occur in CD patients with or without POEMS syndrome.<ref name=clinicalupdate/> CD and POEMS tend to overlap, with roughly 15–24% of POEMS syndrome patients also having CD, with the majority having [[hyaline]] vascular type.<ref name=mjhid/>

==== Sclerotic bone lesions ====
[[Osteosclerosis|Osteosclerotic]] [[lesion]]s have been documented in around 95% of patients. Bone [[lesion]]s can be [[Sclerotic ring|sclerotic]], [[Lytic lesion|lytic]] with sclerotic rims, or a mixture of the two with a soup-bubble look.  About half of the patients have a single bone lesion, whereas the other half have several lesions. The most prevalent locations for bone lesions are the [[pelvis]], [[Vertebral column|spine]], [[Rib cage|ribs]], and [[proximal]] extremities.<ref name=mjhid/>

==== Vascular endothelial growth factor ====
{{main|Vascular endothelial growth factor }}
[[Vascular endothelial growth factor|VEGF]] levels in [[Plasma diagnostics|plasma]] and [[Serum (blood)|serum]] are significantly higher in POEMS patients and correlate with illness activity. [[Osteoblast]]s, [[Bone|bone tissue]], [[macrophage]]s, [[Neoplasm|tumor cells]], [[plasma cell]]s, and [[megakaryocyte]]s all express [[Vascular endothelial growth factor|VEGF]]; both IL1 and IL6 have been proven to increase [[Vascular endothelial growth factor|VEGF]] synthesis. [[Vascular endothelial growth factor|VEGF]] normally targets [[Endothelium|endothelial]] cells and causes an increase in vascular permeability that is both fast and reversible. Increased [[Vascular endothelial growth factor|VEGF]] could explain several clinical features of POEMS, including [[organomegaly]], [[edema]], [[Skin condition|skin abnormalities]], and [[Peripheral neuropathy|neuropathy]], as well as increasing [[Blood vessel|microvascular]] permeability of blood vessels with [[Endoneurial fluid|endoneurial]] edema.<ref name=mjhid/>

=== Minor criteria ===
==== Organomegaly ====
{{main|Organomegaly}}
POEMS syndrome has been associated with [[hepatomegaly]], [[splenomegaly]], and [[lymphadenopathy]] in 50–78% of patients. [[Organomegaly]] is usually minimal, and bulky disease is uncommon.<ref name=mjhid/>

==== Extravascular volume overload ====
{{further|Peripheral edema|Ascites|Pleural effusion|Pericardial effusion}}
80% of POEMS patients are reported to have extravascular volume overload including, [[peripheral edema]], [[ascites]], [[pleural effusion]], and [[pericardial effusion]]. [[Ascites]] and [[peripheral edema]] are more prevalent than [[Pleural effusion|pleural]] or [[pericardial effusion]]s. Extravascular volume overload can cause significant morbidity and corresponds with a lower survival rate.<ref name=mjhid/>

==== Endocrinopathy ====
{{see also|Endocrine disease|Endocrinology|Endocrine system}}
Approximately 84% of POEMS syndrome patients have characteristics of several [[Endocrine disease|endocrinopathies]].  [[Hypogonadism]] is the most prevalent [[Endocrine disease|endocrine disorder]], followed by [[Thyroid disease|thyroid anomalies]], [[Carbohydrate metabolism|glucose metabolism]] defects, and [[adrenal insufficiency]].<ref name=ajh>{{cite journal|first1=Angela|last1=Dispenzieri|title=POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|date=23 May 2019|volume=94 |issue=7 |pages=812–827 |issn=0361-8609|doi=10.1002/ajh.25495|pmid=31012139 |s2cid=128361561 |doi-access=free}}</ref> Men frequently experience [[Erectile dysfunction|impotence]] and [[gynecomastia]]. [[Amenorrhea]] tends to be common in women.<ref name=medscape/> The cause of [[Endocrine disease|endocrinopathy]] is unknown, however [[Vascular endothelial growth factor|VEGF]] may have a role.<ref name=mjhid/>

==== Skin changes ====
{{further|Skin condition}}
About 90–100% of those with POEMS syndrome will experience [[Skin condition|skin changes]]. The most common manifestations are [[hyperpigmentation]] and [[Hemangioma|haemangiomas]]. Other skin abnormalities include thickening, [[hypertrichosis]], acquired facial [[lipoatrophy]], and infiltrating [[livedo reticularis]] with [[necrosis]]. POEMS syndrome can also cause Vascular-type skin changes including [[acrocyanosis]], [[Flushing (physiology)|flushing]], [[hyperaemia]], and [[Raynaud syndrome|Raynaud's phenomenon]]. [[Nail disease|Nail changes]] consist of [[leukonychia]] and [[Nail clubbing|clubbing]].<ref name=clinicalupdate/>

==== Papilledema ====
{{main|Papilledema}}
[[Papilledema]] is often one of the earliest signs of POEMS disease and is usually bilateral.<ref name=mjhid/> Patients tend to be [[asymptomatic]], however, they may report [[headache]]s, brief obscurations of vision, [[scotoma]]ta, large [[Blind spot (vision)|blind spots]], and gradual visual field constriction.<ref name=ashpub/> [[Papilledema]] has been noted in 29–64% of patients and is associated with an unfavorable prognosis.<ref name=mjhid/>

==== Hematological alterations ====
{{main|Thrombocythemia|Polycythemia}}
50% of POEMS patients have [[Thrombocythemia|thrombocytosis]] and [[Polycythemia|polyglobulia]] develops in 15% of patients.  Those with [[Thrombocythemia|thrombocytosis]] and [[Polycythemia|erythrocytosis]] are often misdiagnosed with [[Myeloproliferative neoplasm|chronic myeloproliferative disease]] before POEMS syndrome is identified.<ref name=mjhid/>

=== Laboratory findings ===
In addition to tests corresponding to the above findings, such as [[Electromyography|EMG]] for neuropathy, [[CT scan]], [[bone marrow biopsy]] to detect clonal [[plasma cell]]s, [[Serum protein electrophoresis|plasma or serum protein electrophoresis]] to myeloma proteins, other tests can give abnormal results supporting the diagnosis of POEMS syndrome. These included raised blood levels of [[VEGF]], [[thrombocytes]], and/or [[erythrocyte]] parameters.<ref name="pmid28299525"/>

=== Differential diagnosis ===
Patients diagnosed as having Castleman disease but also exhibiting many of the symptoms and signs of POEMS syndrome but lacking evidence of a [[peripheral neuropathy]] and clonal [[plasma cell]]s should not be diagnosed as having POEMS syndrome. They are better classified as having Castleman disease variant of POEMS syndrome. These patients may exhibit high blood levels of the [[interleukin-6]] cytokine and have an inferior overall survival compared to POEMS syndrome patients. Treatment of patients with this POEMS syndrome variant who have evidence of bone lesions and/or myeloma proteins are the same as those for POEMS syndrome patients. In the absence of these features, treatment with [[rituximab]], a monoclonal antibody preparation directed against B cells bearing the [[CD20]] antigen, or [[siltuximab]], a monoclonal antibody preparation directed against interleukin-6, may be justified.<ref name="pmid28299525"/><ref name=ajh/>

== Treatment ==
As reported by Dispenzieri ''et al.''<ref name=ajh/> [[Mayo Clinic]] treatment regimens are tailored to treat the clinical manifestations and prognosis for the rate of progression of the POEMS syndrome in each patient. In rare cases, patients may have minimal or no symptoms at presentation or after successful treatment of their disorder. These patients may be monitored every 2–3 months for symptoms and disease progression. Otherwise, treatment is divided based on the local versus systemic spread of its clonal plasma cells. Patients with one or two plasmacytoma bone lesions and no clonal plasma cells in their bone marrow biopsy specimens are treated by surgical removal or [[radiotherapy]] of their tumors. These treatments can relieve many of the syndromes clinical manifestations including neuropathies, have a 10-year overall survival of 70% and a 6-year progression-free survival of 62%. Patients with >2 plasmacytoma bone lesions and/or increases in bone marrow clonal plasma cells are treated with a low-dose or high-dose chemotherapy regimen, i.e. a [[corticosteroid]] such as [[dexamethasone]] plus an [[alkylating antineoplastic agent|alkylating agent]]s such as [[melphalan]]. Dosage regimens are selected on the basis of patient tolerance. Hematological response rates to the dexamethasone/melphalan regimens have been reported to be in the 80% range with neurological response rates approaching 100%. Patients successfully treated with the high-dose dexamethasone/melphalan regimen have been further treated with [[autologous stem cell transplantation]]. In 59 patients treated with the chemotherapy/transplantation regimen, the Mayo Clinic reported progression-free survival rates of 98%, 94%, and 75% at 1, 2, and 5 years, respectively.<ref name=ajh/>

Other treatment regimens are being studied. [[Immunomodulatory imide drug]]s such as [[thalidomide]] and [[lenalidomide]] have been used in combination with [[dexamethasone]] to treat POEMS syndrome patients. While the mechanism of action of these immunomodulators are not clear, they do inhibit the production of cytokines suspected of contributing to POEMS syndrome such as [[VEGF]], [[TNFα]], and [[Interleukin 6|IL-6]] and stimulate [[T cells]] and [[NK cells]] to increase their production of [[interferon gamma]] and [[interleukin 2]] (see [[Immunomodulatory imide drug#Mechanism of action|immunomodulatory imide drug's mechanism of action]]). A double blind study of 25 POEMS syndrome patients found significantly better results (VEGF reduction, neuromuscular function improvement, quality of life improvement) in patients treated with thalidomide plus dexamethasone compared to patients treated with a thalidomide placebo plus dexamethasone.<ref name="pmid28299525"/>

Since VEGF plays a central role in the symptoms of POEMS syndrome, some have tried [[bevacizumab]], a [[monoclonal antibody]] directed against VEGF. While some reports were positive, others have reported [[capillary leak syndrome]] suspected to be the result of overly rapid lowering of VEGF levels. It therefore remains doubtful as to whether this will become part of standard treatment for POEMS syndrome.<ref>{{cite journal |vauthors=Samaras P, Bauer S, Stenner-Liewen F, etal |title=Treatment of POEMS syndrome with bevacizumab |journal=Haematologica |volume=92 |issue=10 |pages=1438–9 |year=2007 |pmid=18024383 |doi=10.3324/haematol.11315 |doi-access=free }}</ref>

== History ==
R. S. Crow, working in [[Bristol]], first described the combination of osteosclerotic myeloma, polyneuropathy and various unusual features (such as pigmentation and clubbing) in two patients aged 54 and 67, in 1956.<ref>{{cite journal |doi=10.1136/bmj.2.4996.802 |author=Crow RS |title=Peripheral neuritis in myelomatosis |journal=Br Med J |volume=2 |issue=4996 |pages=802–4 |year=1956 |pmid=13364332 |pmc=2035359}}</ref>

== References ==
{{reflist}}

{{Medical resources
| ICD11           = {{ICD11|2A83.Y}}
| ICD10           = {{ICD10|D|47|7}}
| ICD9            = {{ICD9|273.8}}
| ICDO            =
| OMIM            =
| MeshID          = D016878
| DiseasesDB      = 29226
| SNOMED CT       = 79268002
| Curlie          =
| MedlinePlus     =
| eMedicineSubj   = derm
| eMedicineTopic  = 771
| PatientUK       =
| NCI             =
| GeneReviewsNBK  =
| GeneReviewsName =
| NORD            =
| GARDNum         = 7411
| GARDName        = POEMS syndrome
| RP              =
| AO              =
| WO              =
| OrthoInfo       =
| Orphanet        = 2905
| Scholia         =
| OB              =
}}

{{Immunoproliferative disorders}}
{{Multiple abnormalities}}

[[Category:Endocrine diseases]]
[[Category:Neurological disorders]]
[[Category:Rare syndromes]]
[[Category:Syndromes affecting the nervous system]]
[[Category:Genodermatoses]]