Poems syndrome C90.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 28.12.2022

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Crow Fukase Syndrome; PEP Syndrome; Shimpo syndrome; Takatsuti Syndrome

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Scheinker 1938

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Acronym for:

  • Polyneuropathy
  • Organomegaly
  • Endocrinopathy
  • Monoclonalgammopathy
  • Skinlesions

The prominent clinical presentation is of moderately active osteosclerotic multiple myeloma with monoclonal gammopathy associated with chronic demyelinating polyneuropathy. Skin lesions are observed in approximately 70% of patients and may be monitor signs of underlying systemic disease (see also under Monoclonal Gammopathies of Cutaneous Significance).

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Increased production of VEGF is associated with the development of angiomas and hypertrichosis.

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Mainly occurring in middle-aged patients (40-50 years).

Clinical features
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Diffuse and circumscribed hyperpigmentations, multiple capillary hemangiomas (22-44% of patients; the clinical findings are identical with the findings of"senile angioma"), which are histologically characterized by a glomeruloid structure. Furthermore, diffuse solid swellings of the extremities, scleroderma-like skin thickenings, if necessary sclerodactyly, vasculitis of the small vessels, hypertrichosis and rarely also nail changes(leukonychia) are found.

The organ manifestations that significantly influence the clinical course of POEMS syndrome are defined as follows:

  • Extravascular volume overload (pericardial effusion, ascites - 50 to 70% of patients)
  • Organ megaly (splenomegaly, hepatomegaly in 50% of patients each)
  • Osteosclerotic and/or osteolytic bone lesions (about 20% of patients)
  • Endocrinopathies (diabetes mellitus, hypothyroidism)
  • Sensory polyneuropathies

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The hemangiomas are characterized by a characteristic histological structure as glomeruloid hemangiomas (reminiscent of glomerula of the kidney).

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Dermatological Symptoms:

  • Hyperpigmentation
  • Hypertrichosis
  • multiple capillary hemangiomas
  • Diffuse, firm swellings of the extremities
  • Scleroderma-like thickening of the skin
  • Sclerodactyly
  • Vasculitides of the small vessels
  • Leukonychia

Organ manifestations:

  • MGUS
  • Extravascular volume overload (pericardial effusion, ascites)
  • organ megaly (splenomegaly, hepatomegaly, lymphadenopathy)
  • osteosclerotic and/or osteolytic bone lesions (about 20% of patients)
  • endocrinopathies (diabetes mellitus, hypothyroidism)
  • Sensory polyneuropathies

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Treatment of the underlying disease, radiotherapy, chemotherapy. Cooperation with internists and neurologists.

In patients with dominant sclerotic plasmacytoma, first-line therapy is radiation.

Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and patients in whom the disease progresses 3 to 6 months after completion of radiotherapy should receive systemic therapy. Corticosteroids are transient, but alkylators are the mainstay of treatment, either in the form of low-dose conventional therapy or high-dose treatment with stem cell transplantation.

Lenalidomide is a promising therapy with manageable toxicity.

Thalidomide and bortezomib are also effective, but their benefits must be weighed against the risk of worsening peripheral neuropathy. Early recognition and initiation of both supportive care and therapy directed against plasma cells lead to the best outcomes (Dispenzieri A 2021).

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There is a close association between POEMS syndrome and ASEOP syndrome.

Case report(s)
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Case report varied according to Yuri Tet al. (2008) .

A 64-year-old Japanese woman with idiopathic thrombocytopenic purpura, was treated long-term with prednisolone. During the course of steroid withdrawal, she developed enlargement of the parotid gland and swelling of the cervical lymph nodes with multiple dome-shaped red papules on the trunk and upper limbs. On admission, the patient was found to have numbness in the lower limbs (polyneuropathy), lymph node swelling, elevated blood glucose levels, Bence Jones protein kappa in the urine (M protein). On the skin, there were multiple hemangiomas in addition to areal hyperpigmentation and hypertrichosis. The overall symptomatology was consistent with a polyneuropathy-organomegaly-endocrinopathy-M-protein skin abnormality syndrome (POEMS). The patient also had peripheral edema, ascites, and pleural effusion.

Histo: The glomeruloid hemangiomas exhibited intravascular capillary growth composed of conglomerates of capillaries resulting in structures resembling renal glomeruli. The cells within the capillary loops were lined by endothelial cells with little cytoplasm (CD31(+)/CD34(+)/CD68(-)/CD105(+)/UEA-1(+), whereas the outer surfaces of the loops were either covered by swollen endothelial cells containing PAS- and immunoglobulin-positive eosinophilic hyaline globules (CD31(+)/CD34(-)/CD68(-/+)/CD105(-)/UEA-1(-)) or cells without globules. These two phenotypically distinct endothelial cells were separated by alpha-smooth muscle actin-positive pericytes. Pericytes and endothelial cells covering the outer surface of the loops were bounded by a basement membrane. Biopsy of the parotid gland and lymph node revealed Sjögren's syndrome and Castleman's disease of the hyaline vascular type, respectively.

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  1. Bardwick PA et al (1989) Plasmacell dyscraisia with polyneurophaty, organmegaly, endocrinopathy, M protein, and skin changes: The POEMS syndrome. Medicine 59: 311-322
  2. Caramaschi P et al (2003) A case of acquired partial lipodystrophy associated with POEMS syndrome. Rheumatology (Oxford). 42: 488-490
  3. Dispenzieri A (2019) POEMS syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol 94: 812-827.
  4. Dispenzieri A (2021) POEMS syndrome: 2021 update on diagnosis, risk-stratification, and management. Am J Hematol 96:872-888.

  5. Elling E et al (2008) Schnitzler syndrome: chronic urticaria and monoclonal gammopathy-an autoinflammatory syndrome. JDDG 6: 626-631
  6. Glazebrook K et al (2015) Computed tomography assessment of bone lesions in patients with POEMS syndrome. Eur Radiol 25:497-504
  7. Kang K et al (2003) POEMS syndrome associated with ischemic stroke. Arch Neurol 60: 745-749
  8. Marinho FS et al (2015) CutaneousManifestations in POEMS syndrome: Case Report and Review. Case Rep Dermatol 7:61-69
  9. Obermoser G et al (2003) Absence of human herpesvirus-8 in glomeruloid haemangiomas associated with POEMS syndrome and Castleman's disease. Br J Dermatol 148: 1276-1278
  10. Schaller M (2001) Improvement of cutaneous manifestations in POEMS syndrome after UVA1 phototherapy. J Am Acad Dermatol 45: 969-970
  11. Scheers C (2002) POEMS syndrome revealed by multiple glomeruloid angiomas. Dermatology 204: 311-313
  12. Yuri Tet al (2008) Glomeruloid hemangioma. Pathol Int 58: 390-395.


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Last updated on: 28.12.2022