Poems syndrome C90.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.08.2021

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Synonym(s)

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

Definition
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Acronym for:

  • polyneuropathy
  • Organomegaly
  • Endocrinopathy
  • Monoclonal Gammopathy
  • Skin(skin) changes

The outstanding clinical picture shows a moderately active osteosclerotic multiple myeloma with monoclonal gammopathy associated with chronic demyelinating PNP. Skin lesions are observed in about 70% of patients and may be monitoring signs of the underlying systemic disease.

Manifestation
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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

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

Diagnosis
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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

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

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.

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

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 05.08.2021