HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Bluish-red discoloration of the acra (ends of the body: peripheral cyanosis); increased in cold and wet weather with a tendency to frostbite; often combined with Cutis marmorata.
Cyanotic discoloration and reduced skin temperature of the acra due to an idiopathic, nerve-autonomous, local venous-capillary vasomotor disturbance of the microcirculation. This leads to a prolonged retention of the oxygen-depleted blood in the atonically dilated venous capillaries and the subsequent venules.
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Occurrence/EpidemiologyThis section has been translated automatically.
Precise data on prevalence are not available. Estimates are 5-10%.
EtiopathogenesisThis section has been translated automatically.
Temperature adaptation disorder of the skin vessels with spastic-atonic dysregulation of the microcirculation: arteriolar spasm, atony/dilatation of the venules. Cause unknown. Familial clustering. In unilateral acrocyanosis, vascular occlusions of the axillary artery must be excluded and treated if necessary (arteriography). Elevated endothelin-1 plasma levels are found in the affected patients.
ManifestationThis section has been translated automatically.
Preferably occurring in young women. Begins predominantly in puberty. Favoured by working in a humid-cold environment and nicotine abuse.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Diffuse, reddish-livid skin discoloration, reduced skin temperature. Mostly hyperhidrosis, possibly doughy, cushion-like swellings, numbness. The numbness can be clearly disturbing and can increase when exposed to cold (acrocyanosis chronica anaesthetica).
Diagnostically typical is the iris diaphragm phenomenon.
Differential diagnosisThis section has been translated automatically.
Central cyanosis due to reduced O2 saturation of the arterial blood as a result of internal diseases (chronic heart and lung diseases, myeloproliferative diseases): skin+tongue/oral mucosa are cyanotic, in contrast to peripheral cyanosis.
Raynaud's syndrome: seizure-like event with vasoconstrictory attacks. The vasoconstrictor phase is followed by a phase of actocynosis.
Cryoglobulinemia: mostly livedo images. Detection of cryoglobulins
Livedo syndromes (see there)
Complication(s)This section has been translated automatically.
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
Metz S (2014) Unclear unilateral, painful acrocyanosis-a dermatological case? JDDG 12: 429-431
Incoming links (33)Acroasphyxia; Acroasphyxia; Acrocyanosis; Acrocyanosis chronica anaesthetica; Acroerythrosis indolens bechterew; Acroerythrosis paraesthetica; Acute paronychia; Angiomatosis, diffuse corticomeningeal; Candida paronychia; Cryofibrinogenesis; ... Show all
Outgoing links (9)Candida; Cryoglobulins; Erythrocyanosis crurum puellarum; Iris diaphragm phenomenon; Livedo reticularis; Livedo syndromes (overview); Perniones; Tinea (overview); Verruca vulgaris;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.