yoga book / pathology / erythromelalgie
Contents
Definition
Erythromelalgia is a disorder of vascular regulation in which paroxysmal (episodic) hyperaemia of the hands or feet leads to an unpleasant or painful sensation of heat and red discolouration. It is thought that neuronal and vascular dysfunctions are interrelated. Other names for the condition include erythralgia, erythermalgia, Mitchell’s syndrome and Gerhardt’s syndrome. The attacks last for minutes or even hours, and more rarely for days, and occur with a frequency that varies greatly from person to person, ranging from several times a day to once every few months. Skin that is normally pale appears visibly reddened, whilst darker skin appears even darker. The condition usually affects both sides of the body. Rarely, the symptoms spread slightly proximally or affect the face.
Attacks can be triggered by high ambient temperatures, for example when the temperature reaches 29°. In addition to exposure to high temperatures, there are other triggers such as physical exertion, standing/walking/running, high humidity, hot or spicy food, warm air from radiators or the warm air from a hairdryer. Stress, caffeine and alcohol can also trigger attacks. In some cases, ambient temperatures as low as around 15 °C are sufficient to trigger an attack. The condition may develop gradually or occur suddenly.
Immersing the affected limb in ice-cold water alleviates the attack, but carries a risk of fissures; moderate cooling is a more suitable option. In this respect, this condition behaves similarly to Raynaud’s disease, which is triggered by exposure to cold or even simply by cool temperatures, but without its ‘tricolour’ phenomenon, or to Flammer’s disease. The symptoms may be mild or may worsen to the point of causing disability and occupational incapacity or inability to work. The feet are affected more frequently than the hands.
The condition usually develops after the age of 20; rarely does it occur as an inherited disorder immediately after birth. Symptoms vary considerably from person to person, even when the underlying genetic mutation is identical. In addition to the primary form, erythromelalgia may also occur secondarily in association with a number of other conditions; in such cases, it is not uncommon for it to appear several years before the underlying condition is diagnosed, although isolated acral hyperthermia that improves with cooling is a characteristic diagnostic feature. In cases of erythromelalgia, generalised vasomotor dysfunction may be present, in the context of which Raynaud’s disease may also occur, possibly in the other limb; for example, the feet may be affected by erythromelalgia, whilst the hands are affected by Raynaud’s disease. However, even this limb may, between attacks, feel cold and exhibit acrocyanosis, or be affected by Raynaud’s disease.
As erythromelalgia often precedes a myeloproliferative syndrome by several years, regular blood tests are recommended.
Epidemiology
The condition is quite rare. In the USA, the incidence in 2009 was 3 per 100,000, with a male-to-female ratio of approximately 3:1. The diagnosis is usually not made until later in life; in this study, the average age was 61.
Cause
- Primary: inherited in an autosomal dominant pattern (genetic point mutations)
- secondary (see predispositions)
- Erythromelalgia-associated poxvirus (ERPV), e.g. in China
- drug-induced: e.g. nifedipine, bromocriptine
Predisposing
- secondary form:
- myeloproliferatives Syndrom (myeloproliferative Neoplasie)
- High blood pressure
- venous insufficiency
- Thrombophlebitis
- Diabetes mellitus
- Gicht
- Multiple sclerosis
- Rheumatoid arthritis
- Systemic lupus erythematosus (SLE)
- Lichen sclerosus
- Spinal cord conditions
- Poisoning: mercury, mushrooms
- sciatica
Diagnosis
- Clinically: on visual inspection and on palpation, hyperthermia and redness
- Outside of a seizure: Seizure triggered by exposure to heat
Symptoms
- Trias: redness, warmth, a burning sensation
- Episodic, painful overheating and redness of the extremities. The pain may feel like a burning or throbbing sensation; it is sometimes described as itchy or like a prickling sensation, and can reach an intensity of up to NRS 10.
- where applicable, tenderness in the affected limb
- moderate swelling caused by hyperperfusion
- rare: persistent pain without concomitant erythema
Therapy
- Preventative: Avoiding exposure to heat
- Ruhe
- Elevating the affected limbs
- Cold therapy (no ice!)
- primary form: gabapentin
- Secondary form: treatment of the underlying condition. In the case of myeloproliferative syndrome: acetylsalicylic acid
- Various medicines have helped individual patients, but none has so far been effective in all cases
Complications
- psychological disorders, depression
- a wide range of limitations, reduced opportunities for personal fulfilment
- occupational disability, where applicable
DD
- peripheral neuropathies
- post-traumatic reflex dystrophy
- post-stroke shoulder-hand syndrome
- Causalgia
- Fabry disease
- Sudeck’s disease (CRPS 1)
- Burning feet Syndrom