acrocyanosis


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Acrocyanosis

 

Definition

Acrocyanosis is a decrease in the amount of oxygen delivered to the extremities. The hands and feet turn blue because of the lack of oxygen. Decreased blood supply to the affected areas is caused by constriction or spasm of small blood vessels.

Description

Acrocyanosis is a painless disorder caused by constriction or narrowing of small blood vessels in the skin of affected patients. The spasm of the blood vessels decreases the amount of blood that passes through them, resulting in less blood being delivered to the hands and feet. The hands may be the main area affected. The affected areas turn blue and become cold and sweaty. Localized swelling may also occur. Emotion and cold temperatures can worsen the symptoms, while warmth can decrease symptoms. The disease is seen mainly in women and the effect of the disorder is mainly cosmetic. People with the disease tend to be uncomfortable, with sweaty, cold, bluish colored hands and feet.

Causes and symptoms

The sympathetic nerves cause constriction or spasms in the peripheral blood vessels that supply blood to the extremities. The spasms are a contraction of the muscles in the walls of the blood vessels. The contraction decreases the internal diameter of the blood vessels, thereby decreasing the amount of blood flow through the affected area. The spasms occur on a persistent basis, resulting in long term reduction of blood supply to the hands and feet. Sufficient blood still passes through the blood vessels so that the tissue in the affected areas does not starve for oxygen or die. Mainly, blood vessels near the surface of the skin are affected.

Diagnosis

Diagnosis is made by observation of the main clinical symptoms, including persistently blue and sweaty hands and/or feet and a lack of pain. Cooling the hands increases the blueness, while warming the hands decreases the blue color. The acrocyanosis patient's pulse is normal, which rules out obstructive diseases. Raynaud's disease differs from acrocyanosis in that it causes white and red skin coloration phases, not just bluish discoloration.

Treatment

Acrocyanosis usually isn't treated. Drugs that block the uptake of calcium (calcium channel blockers) and alpha-one antagonists reduce the symptoms in most cases. Drugs that dilate blood vessels are only effective some of the time. Sweating from the affected areas can be profuse and require treatment. Surgery to cut the sympathetic nerves is performed rarely.

Prognosis

Acrocyanosis is a benign and persistent disease. The main concern of patients is cosmetic. Left untreated, the disease does not worsen.

Resources

Books

Alexander, R. W., R. C. Schlant, and V. Fuster, editors. The Heart. 9th ed. New York: McGraw-Hill, 1998.

Key terms

Sympathetic nerve — A nerve of the autonomic nervous system that regulates involuntary and automatic reactions, especially to stress.

acrocyanosis

 [ak″ro-si″ah-no´sis]
persistent cyanosis of the fingers and hands or the toes and feet, with mottled blue or red discoloration, coldness, and profuse sweating of the digits. It may be seen in newborn infants or during the first weeks of life in response to exposure to cold.

ac·ro·cy·a·no·sis

(ak'rō-sī-ă-nō'sis),
A circulatory disorder in which the hands, and less commonly the feet, are persistently cold and blue; some forms are related to Raynaud phenomenon.
[acro- + G. kyanos, blue, + -osis, condition]

Acrocyanosis

An acquired condition marked by symmetrical cyanosis of the distal (hence, acro-) extremities, with persistent, blue and/or red mottling of the skin of the digits, wrists and ankles, accompanied by profuse sweating and cold extremities.

acrocyanosis

Raynaud sign Clinical medicine An acquired condition marked by symmetrical cyanosis of the extremities, with persistent, blue and/or red mottling of the skin of the digits, wrists and ankles, accompanied by profuse sweating and cold extremities. See Raynaud's phenomenon.

ac·ro·cy·a·no·sis

(ak'rō-sī-ă-nō'sis)
A circulatory disorder in which the hands, and less commonly the feet, are persistently cold and blue; some forms are related to the Raynaud phenomenon.
Synonym(s): Crocq disease, Raynaud sign.
[acro- + G. kyanos, blue, + -osis, condition]

acrocyanosis

Blueness, coldness and sweating of the hands and feet in cold weather, due to spasm of small blood vessels. Acrocyanosis is a feature of RAYNAUD'S DISEASE.

Crocq,

Jean, Belgian physician, 1868-1925.
Crocq disease - a circulatory disorder. Synonym(s): acrocyanosis

Raynaud,

Maurice, French physician, 1834-1881.
Raynaud disease - Synonym(s): Raynaud syndrome
Raynaud gangrene - Synonym(s): Raynaud syndrome
Raynaud phenomenon - spasm of the digital arteries, with blanching and numbness or pain of the fingers, often precipitated by cold.
Raynaud sign - Synonym(s): acrocyanosis
Raynaud syndrome - idiopathic paroxysmal bilateral cyanosis of the digits. Synonym(s): Raynaud disease; Raynaud gangrene; symmetric asphyxia

ac·ro·cy·a·no·sis

(ak'rō-sī-ă-nō'sis)
Circulatory disorder in which the hands, and less commonly the feet, are persistently cold and blue.
[acro- + G. kyanos, blue, + -osis, condition]
References in periodicals archive ?
Failure to detoxify sulfide is due to the absence or malfunctioning of a mitochondrial sulfur dioxygenase, encoded by the ETHE1 gene, which is mutated in EE, and characterized by ethylmalonic and methylsuccinic aciduria and lactic acidemia associated with neurodevelopmental delay and regression, pyramidal and extrapyramidal signs, vascular lesions determining episodes of acrocyanosis, recurrent petechiaea and chronic diarrhea (6-8).Our patients had acrocyanosis, chronic diarrhea and psychomotor delay.
Other transient skin conditions seen are milia (5), vernix caseosa (5), acrocyanosis (3), neonatal acne (3), erythema toixcum neonatorum (1) and neonatal alopecia (1).
(12.) Kurklinsky A, Miller V, Rooke T Acrocyanosis: the flying Dutchman.
Topical propranolol 1% cream was well tolerated, without noticeable side effects (hypotension, bronchospasm, bradycardia, hypoglycemia, acrocyanosis, gastrointestinal and sleep disturbances, or respiratory infections) in any patient.
The condition is associated with ecchymotic predisposition, premature varicose veins, diffuse muscle and joint pain, and orthostatic acrocyanosis. The etiology of POTS in JHS patients is thought to be due to abnormal vascular (venous) elastic connective tissue.
Related health problems include skin and vascular disorders (acrocyanosis, angina pectoris, hypertension, myocardial infarction, mesenteric thrombosis, systemic occlusive arterial disease, bronchiectasis and recurrent bronchi-pneumonia), neuropathy, gastroenteritis, hepatoxicity and hematological abnormalities such as anemia and leucopenia [11].
Score 0 1 2 Appearance Body all Blue at Body all pink blue extremities (no cyanosis) (cyanosis) (acrocyanosis) Pulse Absent Less than Greater than 100bpm 100bpm Grimace No Grimace and Cry or pull away response to weak cry when when stimulated stimulation stimulated Activity None Some flexion Flexed arms and legs that resist extension Respiration Absent Weak, Strong cry irregular, gasping Need for neonatal resuscitation (circle 1): Yes No
For example, flushing and acrocyanosis are relatively normal.
2004; Fledelius 1999), acrocyanosis (Hoegl, Poppinger & Rocken 1999), altered gene expression and impaired tumor surveillance mechanisms (Tran et al.
The World Health Organization has recommended the use of GB in Raynaud's disease (a common and painful condition characterized by episodic digital ischaemia produced by emotion and cold), acrocyanosis, and postphlebitic syndrome (11).
Clues to the diagnosis of cold agglutinin disease include acrocyanosis and Raynaud's phenomenon.
On his fourth day of life, the patient presents high blood pressure and on the seventh day acrocyanosis is evidenced, absence of pulsations and arterial tension in the lower limbs is not present.