CryoproteinemiaD89.1
DefinitionThis section has been translated automatically.
Occurrence of plasma or serum proteins that precipitate as precipitate or gel at low temperatures. Among the cryoproteins, a distinction is made between cryoglobulins, plasma cryoproteins and fibronectin.
EtiopathogenesisThis section has been translated automatically.
Different underlying diseases: myeloma, macroglobulinemia, chronic lymphatic leukemia, chronic liver disease, autoimmune diseases and infections.
LocalizationThis section has been translated automatically.
Especially lower legs and extremities are affected.
Clinical featuresThis section has been translated automatically.
- Raynaud's phenomenon, acrocyanosis, livedo reticularis, petechial haemorrhages, haemorrhagic blisters, ecchymoses or necroses caused by cold, prolonged standing, exertion or medication. After repeated episodes: Brownish hyperpigmentation. In 10% of cases cold urticaria, also cold panniculitis.
- Optional: Disseminated sarcoidosis-like nodules, ulcerated plaques, orange skin infiltrates. Involvement of internal organs.
LaboratoryThis section has been translated automatically.
The unspecific syphilis is false positive. BKS acceleration at 37 °C and decrease at 4 °C.
HistologyThis section has been translated automatically.
Inflammatory vascular changes, intravascular protein precipitation, epidermal necroses, blistering.
DiagnosisThis section has been translated automatically.
Detection of cryoproteins, electrophoresis, precipitation at refrigerator temperature.
TherapyThis section has been translated automatically.
Treatment of the underlying disease. Symptomatic: avoidance of cold. Experiments with plasmapheresis, immunosuppressants, hemodialysis, penicillamine, chlorambucil, chloroquine, possibly anticoagulants.
LiteratureThis section has been translated automatically.
- Jansen TL et al (2003) Transient cryofibrinogenaemia with acral digital necrosis, secondary Raynaud's phenomenon and polyarthritis. Clin Exp Rheumatol 21: 407-408
- Krunic AL et al (2003) Cryoglobulinaemic vasculitis, cryofibrinogenaemia and low-grade B-cell lymphoma. Br J Dermatol 148: 1079-1081
- Kuhl V et al (2001) Intravenous immunoglobulin and prednisolone treatment of cryoglobulinemic polyneuropathy. Neurologist 72: 445-448
- Landthaler M (1980) The cryoproteinemias. dermatologist 31: 633-638
- Paueksakon P et al (2003) Local cryoglobulin deposition in primary central nervous system lymphoma. Hum Pathol 34: 720-724
- Persico M et al (2003) Prevalence and incidence of cryoglobulins in hepatitis C virus-related chronic hepatitis patients: a prospective study. Am J Gastroenterol 98: 884-888
- Strunk J et al (2002) Essential cryoglobulinemic vasculitis with severe peripheral neuropathy and neurogenic muscular atrophy -- inducing remission by cascade filtration. Z Rheumatol 61: 733-739