Raynaud's Syndrome

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Right hand with pronounced Raynaud's phenomenon
Classification according to ICD-10
I73.0 Raynaud's Syndrome
ICD-10 online (WHO version 2019)

Raynaud's phenomenon (named after Maurice Raynaud [ ʁɛno ]) is characteristic of Raynaud's syndrome or Raynaud's disease , an attack-like symmetrical pale of the fingers or toes due to spasmodic constrictions of the blood vessels ( vasospasms ). The vasoconstriction may also affect other acra such as the nose or ears. In the United States, the National Institutes of Health estimates that around 5 to 10 percent of the population suffer from Raynaud's syndrome. Women are five times more likely to be affected than men. In breastfeeding women, the nipples can also be affected, and the nipple turns white while breastfeeding. In the course of this functional vascular disorder , gangrene can occur ( Raynaud's gangrene ).

The disease is named after the French doctor Maurice Raynaud (1834–1881), who first described it in detail in his medical dissertation in 1862. Colloquially it is also called white finger disease or corpse finger , medical names for the symptoms are digitus mortuus (dead finger) or Reilscher finger.

Pathogenesis

The exact pathogenesis has not yet been clarified. Different factors can be involved in the genesis of the disease and trigger different reaction patterns. Normally, the body tries to minimize heat loss in the event of exposure to the cold by activating the autonomic nervous system by directing more blood from the superficial skin vessels into the deeper body veins.

In many cases, Raynaud's syndrome is based on a dysregulation by the sympathetic part of the autonomic nervous system. Alpha-adrenoreceptors cause excessive vasoconstriction of the end arteries ( arterioles ). The blood flow in the affected areas is severely restricted by the vascular spasm that is triggered . Usually these spasms resolve on their own.

Front and back of the same hand at the beginning of a Raynaud phenomenon

Symptoms

The phenomenon to be observed in Raynaud's syndrome usually has three phases in the affected regions:

  1. Ischemia - with pale skin - due to attack-like vascular spasms, the arteries narrow. The result is a significant reduction in blood flow to the acra . If this continues, numbness, a feeling of cold, numbness, decreased mobility and pain occur.
  2. Cyanosis - blue-colored skin - from hypoxia . The veins widen in response to the undersupply of the tissue. The tissue has withdrawn more oxygen from the accumulated venous blood than usual, which is reflected in a bluish discoloration.
  3. Hyperemia - with reddened skin - reactive leads to increased blood flow with reddening and swelling, tingling, throbbing, sensation of heat and pain.

The characteristics of these three phases can vary from case to case. The typical color change of the skin region is also known as the tricolor phenomenon.

If the region is not supplied for a long time or if the disease has persisted for a long time, secondary damage to the vessel walls with subsequent necrosis and gangrene can occur.

to form

Primary Raynaud's Syndrome

A primary Raynaud's syndrome - synonyms are Raynaud's disease or Raynaud's disease - is when the symptoms occur without an identifiable underlying disease. An attack is often triggered by exposure to the cold or psychological stress.

Secondary Raynaud's Syndrome

The secondary Raynaud's syndrome (syn. Raynaud's phenomenon) occurs as a concomitant disease, z. B. at

Diagnosis

The acral oscillography helps to objectify a reduced blood flow by means of laser or light-controlled pulses. In connection with a provocation test, in which the patient is exposed to a warm and / or cold bath, the symptoms and their causes can be narrowed down. In the case of a secondary Raynaud phenomenon, diagnostic clarifications of the underlying disease are necessary.

While the primary Raynaud's phenomenon usually shows symmetrical involvement of both hands, the secondary Raynaud's phenomenon often only occurs on one side.

therapy

Raynaud's syndrome is usually harmless. This can be remedied by warm clothing, body heat in general, keeping fingers and toes warm, exercise and a massage that promotes blood circulation. In addition, both physical and mental training in terms of stress control have proven effective.

Vasodilation is medicated by alpha-receptor blockers or calcium antagonists , such as. B. nifedipine achieved. This treatment has also been tested as effective in nursing mothers whose nipples are affected in the United States. In recent years, prostaglandin E1 (alprostadil) has also been used increasingly .

Should this therapy fail or, in severe cases, be insufficient, consideration should be given to severing the sympathetic nervous system that innervates the affected extremities ( sympathectomy ). The stellate ganglion and the second and third thoracic ganglion are switched off. However, this disrupts sweat regulation and causes local hyperemia . In addition, the operation is not without risk due to the position of the sympathetic trunk on the side of the vertebral bodies in the chest .

In severe cases of secondary Raynaud's syndrome, special apheresis can be used to treat the causative disease, such as an autoimmune disease , and to stimulate blood flow to the tissue.

A more recent attempt at therapy consists in taking sildenafil , which relieves the symptoms through its vasodilating effect. This form of therapy is currently in clinical trials in Germany. Therapeutic also prostacyclin and prostacyclin analogs such. B. Iloprost (trade name Ilomedin ), used to treat Raynaud's syndrome.

See also

Web links

Individual evidence

  1. ↑ a b Jane E. Anderson, Nancy Held, Kara Wright: Raynaud's Phenomenon of the Nipple: A Treatable Cause of Painful Breastfeeding . In: Pediatrics . tape 113 , no. 4 , April 2004, ISSN  0031-4005 , p. e360-e364 , PMID 15060268 .
  2. ^ Herbert Reindell , Helmut Klepzig: Diseases of the heart and the vessels. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 450-598, here: pp. 590 f. ( Raynaud's disease ).
  3. ↑ Maurice Raynaud: De l'Asphyxie Locale et de la Gangrène Symétrique des Extrémités. Thèse pour le doctorat en Médecine. L. Leclerc, Paris 1862.
  4. ↑ Marianne Brydøy et al: Observational Study of Prevalence of Long-term Raynaud-Like Phenomena and Neurological Side Effects in Testicular Cancer Survivors. In: J Natl Cancer Inst. 2009; 101, pp. 1682-1695.