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Achenbach's Syndrome



Hello! I have been away for a while due to my third reinfection with SARS-CoV-2. Probably this time it was some variant of Omicron. Much milder than the original strain and Delta infections - runny nose, sneezing, sore throat and fatigue. I now know precisely when I am infected with Covid-19 as my POTS symptoms really magnify (especially the dizziness on standing). A week after my lateral flow test was negative my Mast Cell Activation Syndrome has kicked in again with burning and itching skin after I eat or drink anything so I am back on a strict low histamine diet.

Anyway last night I went to bed and had a lot of bruises on my body. Widespread spontaneous bruising happened for almost a year after my first infection. I woke up this morning with a bruised, swollen and painful finger which I have also had a few times in the past 2 years (see title photo).


This is most likely Achenbach's Syndrome and is occurring a lot in the Long Covid community. It is definitely something that most doctors will not have heard of. It is also known as paroxysmal finger haematoma - a sudden finger bruise.


Achenbach's syndrome is characterized by the appearance of haematomas, spontaneous or associated with minor trauma, on the palmar side of the fingers. It involves more frequently the fingers of the hands, although there are reports of it occurring on the palms, soles or toes. It is said to occur most commonly in the distal part of the index finger.




It is associated with pain, sensation of pain and oedema on the site of appearance of the hematoma.

It is more common in women. There is a ratio of 7 women for every man affected. Although it can occur at any age, its maximum peak seems to be around 50 years, the frequency of presentation increases in people older than 50 years.

Its etiology is unknown, however, it has been found an association with conditions such as:

  • acrocyanosis (bluish discoloration of the extremities due to decreased amount of oxygen delivered to the peripheral part)

  • gastrointestinal diseases

  • liver and gallbladder diseases

  • migraine

  • systemic lupus erythematosus


It is believed that this condition could have a vascular origin; the capillary fragility associated with age could explain the symptoms presented by patients affected by this condition.


The only systematic review of the subject, published in 2015, included 12 patients aged between 22 and 76 years. The discoloration of the fingers was present in all the patients, while the blue coloration only in 10; 7 had pain, 7 swelling, 3 numbness and 3 itching.

The mean time to symptom resolution was 4 days.

The most frequently affected anatomical region was the palmar aspect of the fingers. All patients were studied with coagulation tests, blood count, sedimentation rate, electrolytes and immunological tests. In some of them, hand ultrasound, X-rays, angiography and biopsy were performed, which were always negative. None of the studies carried out on the patients showed conclusive physiopathological results.


Another study however showed that small vessel disease did exist in some patients with Achenbach Syndrome. This was mostly due to slow flow of the blood to the fingers and toes. Microclots were not determined.




As can be seen from the table above there are many differential diagnoses for Achenbach's Syndrome. I will discuss the most common here.


Raynaud's syndrome, the average age of onset is 14 years, and the cases that start after age 30 are rare. Though I expect less so now with Long Covid. The clinical findings include paleness, cyanosis and redness of the fingers of the hands and feet, secondary to a vasospastic phenomenon related to stress and cold. Achenbach's Syndrome can be associated with Raynaud's Syndrome too.

Peripheral vascular disease differs from Achenbach's syndrome by its predominance in males and an older age of presentation.

In a digital venous thrombosis usually appears as a bluish and painful nodule in the proximal interphalangeal joint, however, the pain is less intense than that experienced by patients with Achenbach's syndrome. Patients with livedo reticularis show a speckled purpuric skin pattern characteristic of this disease.


Please note that the blue coloration on the fingers can be the manifestation of a potentially serious disease. Trauma or alterations in coagulation should be ruled out in the initial evaluation, as well as cyanosis secondary to hypoxemia (low oxygen levels) or methemoglobinemia (carbon monoxide poisoning) It may also be a coloration change induced by cold, by vasospastic disorders such as Raynaud's phenomenon or by acrocyanosis secondary to hematological alterations, drugs, malignant conditions, metabolic, neurological, psychiatric or alimentary disorders or arsenic poisoning. The possibility of thrombosis or peripheral embolism should also be considered. So with that said if you are at all concerned please see your GP/Accident & Emergency urgently.


I personally feel that with the increased appearance and occurrence of Achenbach's Syndrome in the Long Covid community that this is further evidence of impaired circulation by Microclots. It will be interesting to see if any research flows looking into this.


References

https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition--474-articulo-achenbach39s-syndrome-case-report-systematic-S2444440518300104


https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6547318/











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