DefinitionThis section has been translated automatically.
- 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.
ManifestationThis section has been translated automatically.
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Clinical featuresThis section has been translated automatically.
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
HistologyThis section has been translated automatically.
DiagnosisThis section has been translated automatically.
- multiple capillary hemangiomas
- Diffuse, solid swelling of the extremities
- Scleroderma-like thickening of the skin
- Vasculitis of the small vessels
- 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
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Bardwick PA et al (1989) Plasmacell dyscraisia with polyneurophaty, organmegaly, endocrinopathy, M protein, and skin changes: The POEMS syndrome. Medicine 59: 311-322
- Caramaschi P et al (2003) A case of acquired partial lipodystrophy associated with POEMS syndrome. Rheumatology (Oxford). 42: 488-490
- Elling E et al (2008) The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy-a autoinflammatory syndrome. JDDG 6: 626-631
- Glazebrook K et al (2015) Computed tomography assessment of bone lesions in patients with POEMS syndrome. Eur Radiol 25:497-504
- Kang K et al (2003) POEMS syndrome associated with ischemic stroke. Arch Neurol 60: 745-749
- Marinho FS et al (2015) CutaneousManifestations in POEMS Syndromes: Case Report and Review. Case Rep Dermatol 7:61-69
- 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
- Schaller M (2001) Improvement of cutaneous manifestations in POEMS syndrome after UVA1 phototherapy. J Am Acad Dermatol 45: 969-970
- Scheers C (2002) POEMS syndrome revealed by multiple glomeruloid angiomas. Dermatology 204: 311-313
Incoming links (13)Angioma seniles; Castleman lymphoma; Crow fukase syndrome; Hemangioma glomeruloides; Multiple myeloma; Papillary hemangioma; Pep syndrome; Plasma cytosis cutaneous and systemic; Platelet; Polyendocrine autoimmune syndromes; ... Show all
Outgoing links (7)Angioma seniles; Hemangioma glomeruloides; Hypertrichoses; Leukonychie; Multiple myeloma; Paraproteinemic skin diseases; Sclerodactyly;
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