Raynaud's syndrome I73.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 16.11.2022

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Synonym(s)

Corpse finger; Dead finger; digitus mortuus; Extremities gangrene symmetrical; Primary Raynaud's syndrome; Raynaud phenomenon; Raynaud's disease; Raynaud's phenomenon; Raynaud's symptom complex; Raynaud's Syndrome; Reilscher finger; RS; Secondary Raynaud's syndrome; White Finger Disease

History
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Raynaud, 1862

Definition
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Seizure-like vasospasms associated with numbness (45% of patients), tingling paresthesias (20% of patients) and pain (60% of patients), mostly symmetrical, but also asymmetrical or isolated, functional, digital vasospasms, which can be primarily idiopathic or secondary and can be relieved by heat or medication.

The term "Raynaud's phenomenon" is to be equated with Raynaud's syndrome and does not require a separate assessment.

Classification
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Primary Raynaud's syndrome (>50%) - conceptually equivalent to Raynaud's disease. Vasospasms of the fingers triggered by cold and emotional stress up to max. 30 min. duration.

Secondary Raynaud's syndrome (Raynaud's phenomenon): similar symptoms with symmetrical/asymmetrical affection mostly associated with diseases of the so-called collagenoses.

Paraneoplastic Raynaud's syndrome also belongs to this group. It is associated with lung carcinomas, carcinomas of the ovaries and uterus (Lai TS et al. 2020; Lokineni S et al. 2021).

Occurrence/Epidemiology
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The prevalence in the German population is about 8-10%; in Sweden about 20%, in Switzerland about 20-30%.

Etiopathogenesis
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Vasospasms triggered by cold, emotional stress, local compression phenomena.

Manifestation
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Occurring between the ages of 20 and 50 (average age 36). Rare are first manifestations already in childhood or after the age of 65. Women are 5 times more frequently affected than men.

Localization
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Especially the Digiti II-V of the hand are affected, less often also the toes. The thumbs are usually left out, as are the back of the hand and palms.

Clinical features
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Mostly bilateral seizure symptoms with inital cyanosis followed by seizure-like white coloration which turns into an exuberant redness. In 1/3 of the patients, however, only a seizure-like cyanosis or only a white discoloration of the fingers occurs.

Differential diagnosis
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Permanent acral ischemia without seizure character is to be distinguished.

Embolism (duration of ischemia > 30 min.)

PAVK (DD and one of the causes of secondary Raynaud's syndrome)

Isolated acrocyanosis (painless, cyanotic discoloration of the fingers that does not occur in attacks)

Systemic sclerosis of the acroscleroderma type (besides vasospasms of the fingers flat digital swellings and inudurations, sclerotic nail folds, other signs of PSS with antibody detection/centromer-Ak; SCL70-Ak)

General therapy
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  • Clarification and if necessary treatment of an underlying disease. Otherwise symptom- and phase-oriented prophylactic measures: protection against the effects of cold, wearing warm clothing, if necessary also pocket warmers or heated gloves.
  • Smoking ban (vasoconstrictive effect of nicotine).
  • No prescription of pharmaceuticals containing clonidine, ergotamine or epinephrine.
  • Physiotherapy: robotic measures such as alternating warm hand baths with room temperature (not too cold!) and 37 °C. Alternative finger exercises, e.g. fango kneading or kneading of warmed millet (whole grains!) with a few drops of olive oil.
  • Some patients can also increase the finger temperature by bio-feedback exercises or autogenic training.

Notice! Physical therapies such as alternating warm hand baths, fango kneading, kneading warm millet are helpful in Raynaud's symptoms!

External therapy
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Isosorbide dinitrate ointment formerly Isoket ointment, this is withdrawn from the market, therefore, if necessary, magistral prescription or alternative preparation Diltiazem, corresponding to chronic anal fissure, see there. The vasodilating creams can also lead to lowering of blood pressure, headache, but less pronounced as in systemic vasomodulatory therapy. Use as a monotherapeutic or in addition to internal therapy.

As a magistral formulation e.g. 0.2% nifedipine cream (DAC base cream). It should be noted that the preparation should be done in a darkened room due to light sensitivity, which is why not all pharmacies offer this formulation.

For minor injuries, early disinfecting measures such as polyvidone-iodine (e.g., Braunovidon ointment).

Warm kerosene baths of the hands are considered pleasant (note: a simple and inexpensive paraffin-containing body oil is sufficient for this; bath time 5-8min.).

Internal therapy
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  • Use of vasoactive substances.
    • Calcium antagonists (considered the gold standard): 1st choice therapy is nifedipine (e.g. Adalat 5) 5-15 mg/day p.o. as monotherapy or in combination with pentoxifylline (e.g. Trental) 600 mg/day p.o.; increase the dose of nifedipine up to 3-4 times/day 10 mg p.o. Cave! Orthostatic dysregulations with nifedipine! Long-term administration of pentoxifylline favours skin bleeding!
    • Alternatively Diltiazem (e.g. Dilzem Tbl.) 60-120 mg/day p.o. or Verapamil (e.g. Isoptin 80) 240-320 mg/day p.o.
  • Other possible therapy regimens:
    • ACE inhibitors and angiotensin 1 receptor antagonists: Several studies with the ACE inhibitors captopril (25 mg/day) and enalapril (20 mg/day) showed partly good, partly contradictory results. Losartan (angiotensin 1 receptor antagonist) had an effect comparable to 40 mg nifedipin/day in a randomised trial.
    • Alpha 1 receptor blocker: Prazosin (e.g. Minipress) initially creeping in 1 mg p.o. at night, possibly additionally in the morning, slowly increasing to maintenance dose of 4 mg/day.
    • Prostacycline (level of evidence A): e.g. iloprost (ilomedin) 0.5-2.0 ng/kg bw/min. The daily recommended infusion duration is 6-8 hours. Therapy duration: 3-5 days. Therapy cycles are repeated after 3 months.
    • Prostavasin (data unclear): In case of manifest or imminent gangrene! Effective but complex procedure to improve the acral blood circulation. Dosage: Prostaglandin E1 (e.g. Prostavasin) 20 μg/hour i.v. over 3 hours
    • Estrogens: In case of worsening of symptoms during menses and during menopause, peroral administration of estrogens (e.g. Trisequens) by gynaecologists.
    • Endothelin antagonists ( bosentan): Hopeful approach, currently in multicenter study. Dosage: Tracleer 2 times/day 125 mg p.o.
    • PDE-5 inhibitor: In severe cases of refractory acral ulcerations, oral sildenafil (Viagra) at a dose of 20-80 mg/day may be considered. In individual cases iloprost can be combined with sildenafil.

Operative therapie
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Partial sympathectomy (early therapy of choice) is no longer used, favourable effect in 25% of cases.

Tables
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Contributory causes of secondary Raynaud's syndrome:

  • Connective tissue diseases
    • Progressive systemic scleroderma, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, dermatomyositis/polymyositis
  • Arterial occlusive diseases
    • Arteriosclerosis, Endangiitis obliterans, Polyarteritis nodosa, Embolisms, Thromboses
  • Shoulder girdle arm syndromes
    • Cervical rib syndrome, 1st rib syndrome, scalenus syndrome, costoclavicular syndrome, hyperabduction syndrome, pectoralis minor syndrome, malposition syndrome, narrow upper thoracic aperture syndrome, Klippel-Feil syndrome, combination forms
  • Hematogenous diseases
    • Cold agglutinins, cold hemolysins, cryoglobulinemia, macroglobulinemia (Waldenström), paroxysmal hemoglobulinuria, hyperviscosity syndrome, thrombocytosis, polycythemia, thrombotic microangiopathy
  • Neurological diseases
    • Neuritis, poliomyelitis, multiple sclerosis, syringomyelia, nucleus pulposus prolapse, spinal tumors, carpal tunnel syndrome, hemiplegia
  • Intoxications
    • ergot alkaloids (ergotism), fungal toxins (folding tintling), vinyl chloride derivatives (vinyl chloride disease), trichloroethylene
  • Chronic occupational trauma
    • Vibration syndromes when working with jackhammers, power saws, walking on crutches, etc.
  • Trauma
    • Local vascular injuries, post-traumatic, cold damage
  • Medicinal
    • Clonidine, sympathomimetics, ACE inhibitors, hormonal anticonceptives, beta receptor blockers, secale alkaloids (ergotism), bleomycin, vincristine, ciclosporin
  • Other
    • surgery, Sudeck's atrophy, dialysis

Phytotherapy external
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From a naturopathic point of view, ofiizine rosemary oil is recommended, which is applied to the affected hands several times a day as an externum (by Schoen-Angerer T et al. 2018).

Literature
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  1. Belch J et al. (2017)ESVM guidelines - the diagnosis and management of Raynaud's phenomenon.Vasa 46:413-423.
  2. Bowling JC et al (2003) Raynaud's disease. Lancet 361: 2078-2080
  3. Caccavo D et al (2003) Raynaud's phenomenon and antiphospholipid antibodies in systemic lupus erythematosus: is there an association? Ann Rheum Dis 62: 1003-1005
  4. Coffmann JD et al (1989) International study of ketanserin in Raynaud's phenomenon. Am J Med 87: 264-268
  5. Cohen LE et al (1989) Prostaglandin infusion therapy for intermittent digital ischemia in a patient with mixed connective disease. Case report and review of the literature. J Am Acad Dermatol 20: 893-897.
  6. Hummers LK et al (2003) Management of Raynaud's phenomenon and digital ischemic lesions in scleroderma. Rheum Dis Clin North Am 29: 293-313.
  7. Kallenberg CG et al (1987) Nifedipine in Raynaud's phenomenon: relationsship between immediate, short term and longterm effects. J Rheumatol 14: 284-290
  8. Lai TS et al (2020) Paraneoplastic Raynaud's phenomenon associated with metastatic ovarian cancer: A case report and review of the literature. Gynecol Oncol Rep 33:100575

  9. Lokineni S et al (2021) Paraneoplastic Raynaud's phenomenon as an initial manifestation of lung cancer? Eur J Case Rep Intern Med 8:002690

  10. Mayser P et al (2003) Persistent skin reaction and Raynaud's phenomenon after a sting by Echiichthys draco (great weever fish). Dermatologist 54: 633-637
  11. Raynaud AGM (1862) De l?asphyxie locale et de la gangrène symétrique des extrémités. Doctoral thesis, Rignoux, Paris
  12. Raynaud M (1888) On asphyxia and symmetrical gangrene of the extremities 1862 and new researches on the nature and treatment of local asphyxia of the extremities 1874. Translated by T. Barlow In: Selected Monographs, New Sydenham Society, London, pp. 1-199.
  13. Riemekasten G (2005) Recommendations of the German Society of Rheumatology for the treatment of Raynaud's syndrome and acral ulcerations. Z Rheumatol 64: 90-102
  14. Sunderkötter C et al (2006) Raynaud's phenomenon in dermatology. Dermatol 57: 927-942
  15. Suter LG et al (2005) The incidence and natural history of Raynaud's phenomenon in the community. Arthritis Rheum 52: 1259-1263
  16. Von Schoen-Angerer T et al (2018) Effect of topical rosemary essential oil on Raynaud's phenomenon in systemic sclerosis.Complement Ther Med 40:191-194.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 16.11.2022