Poems syndrome C90.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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:

  • Hyperpigmentations
  • Hypertrichosis
  • multiple capillary hemangiomas
  • Diffuse, solid swelling of the extremities
  • Scleroderma-like thickening of the skin
  • Sclerodactyly
  • Vasculitis of the small vessels
  • Leukonychie

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.

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) The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy-a 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 Syndromes: 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

Disclaimer

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

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Last updated on: 29.10.2020