Acrocyanosis I73.81; R23.0;

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acroasphyxia; Acrocyanosis; Cyanosis; Peripheral cyanosis

History
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Crocq 1896

Definition
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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.

Occurrence/Epidemiology
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Precise data on prevalence are not available. Estimates are 5-10%.

Etiopathogenesis
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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.

Manifestation
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Preferably occurring in young women. Begins predominantly in puberty. Favoured by working in a humid-cold environment and nicotine abuse.

Localization
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Hands, feet, possibly also forearms, lower legs, nose, cheeks, ears, gluten.

Clinical features
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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 diagnosis
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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.

Erythrocyanosis crurum puellarum

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)
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Acrocyanosis is a favourable factor for the development of pernions, verrucae vulgaris, fungal infections, candida, among others.

Therapy
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Cold protection, vascular training through physical measures such as alternating baths, sauna, massages, sports activities. Strict abstinence from nicotine. If necessary, external application of hyperemic substances such as nicotinic acid derivatives: e.g. Rubriment ointment 2-3 times/day.

Progression/forecast
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Harmless disorder; improvement or disappearance in the 3rd - 4th decade of life.

Literature
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Metz S (2014) Unclear unilateral, painful acrocyanosis-a dermatological case? JDDG 12: 429-431

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