Achenbach Syndrome


What is Achenbach Syndrome?

Achenbach Syndrome is a paroxysmal hemorrhagic benign condition. Achenbach Syndrome occurs as a bruise on hands or fingers.Treatment recommendation is minimal as Achenbach Syndrome is a self-limiting condition. Alternatively, Achenbach’s syndrome is also known as “finger apoplexia” or “paroxysmal hand hematoma”.

The name of the Achenbach syndrome denoted the first describer name. In 1958, Achenbach Syndrome was first demonstrated by Walter Achenbach. Walter Achenbach described that onset of this syndrome is sudden and recurrent episodes of bleeding and bruising of the palm of the hand or fingers affects usually middle aged women1,2.

Achenbach Syndrome Picture 1


Clinical Presentation

The lesions associated with Achenbach syndrome usually occur at the index and middle finger. The onset of the disease is sudden and spontaneous, however insignificant traumatic injury may sometimes trigger the episode.

The affected individual often complains following symptoms:

  • Itching or burning sensations in the finger and subsequently appear bruise at the palmar aspect of the finger.
  • Profuse bleeding appears within few hours after prodromal symptoms like pain or tingling sensation develop.
  • Local pressure application can cause bleeding cessation and follow by discoloration.
  • The affected finger gradually turns to become inflamed, pale and cold, without involving nail bed and fingertips.
  • The whole episode usually lasts between 4 to 6 days, after the onset of initial symptoms.
  • No bleeding evidence occur at the distant organ.

The every individual affected with Achenbach syndrome has variable recurrence rate. However, recurrence rate may increase with the association of co-morbidities like gastrointestinal disorders, gall bladder disease, migraines, and acrocyanosis1,2,3.

Causes

The precise result of Achenbach syndrome is not clearly understood, it is still under a research process. However, depending upon the different case studies findings the possible approaches are: subcutaneous bruising, considerably inhibited blood flow to digits underlying cause of Achenbach syndrome.

Few case reports also showed capillary resistance reduction with normal capillary histology present in some affected individuals.


In addition, experts also deduced that anti-migraine medication like ergotamine may have a role in inducing a vasospastic reaction by perusing the microvascular digital flow1,2.

Co-morbidities

Study article showed Achenbach’s syndrome has an association with co-morbidities, such as joint pain, cholelithiasis, Raynaud’s syndrome, migraine, thyroid abnormalities and allergy. However, no pathophysiological reactions found yet between the Achenbach’s syndrome and the mentioned co-morbidities1,2.

Diagnosis

The diagnosis of Achenbach’s syndrome usually depends on an assessment of clinical presentation. The local affected area monitoring usually shows the expansion of the discoloration is restricted only to the volar portion of the finger and usually does not occupy the tip of the finger.

The investigative tests including the hematological report, including hemogram, clotting time, bleeding time, coagulation profile, other biochemical parameters are within normal limit. Other routine examinations like Doppler test, Electrocardiogram report provide a normal result.

Histopathological examination, which is only conducted in research purpose showed that the lesion is acanthotic epidermis with mild hyperkeratosis. The external dermis layer has dilated vascular pores covered with a single layer of endothelial cells along with scanty lymphocytic infiltrate. There is no evidence of deep dermis layer abnormality.

However, these clinical investigations are rarely ordered, as through assessment of medical history and self-limiting approach of Achenbach’s syndrome is enough to manage this syndrome1,2.

Risk for Misdiagnosis

Achenbach syndrome has a chance to misdiagnosis, as the similar type of symptoms like bluish discoloration of the finger is also classic symptoms of connective tissue diseases, occlusive arterial diseases, nerve lesions, using of some medications, including ergotamine, and thoracic inlet syndrome.

But these conditions have also associated with simultaneous involvement of other fingers, systemic involvement like vascular abnormalities, nerves problem, abnormal composition of blood etc.

“Painful bruising syndrome” or “autoerythrocyte sensitization syndrome” are also had similar initial symptoms. Therefore, distinguishing of Achenbach syndrome may difficult at the initial stage.

However, in “Painful bruising syndrome” or “autoerythrocyte sensitization syndrome”, bruising is not restricted to fingers, but expand into lower limbs and trunk. In addition, psychiatric complaints are strongly associated with “Painful bruising syndrome” or “autoerythrocyte sensitization syndrome”1,2.

Management

Achenbach Syndrome is a self-limiting condition, so usually does not require any medical treatment. But proper awareness about its benignity is very important for both patient and clinician.

Proper counseling helps to reduce anxiety, which may develop due to pain and movement restriction. Counseling also provides awareness and avoidance for proceeding needless invasive investigations, which can act as triggering factors1,2.

Pictures

Achenbach Syndrome Picture 2

Achenbach Syndrome Picture 3

Achenbach Syndrome Picture 4


References

  1. S. Kämpfen, D.D. Santa, C. Fusetti; A Painful Blue Thumb: A Case of Achenbach’s Syndrome; http://dx.doi.org/10.1016/j.ejvsextra.2005.07.004; Retrieve from
  2. http://www.sciencedirect.com/science/article/pii/S153331670500083X
  3. Pranshu Mishra, Atul Jain, Sumit Sen, and Banashree Majumdar; Recurrent Bleeding and Bruising Over Little Finger: A Diagnostic Conundrum!; Indian J Dermatol. 2015 Nov-Dec; 60(6): 632–633.
  4. doi:  10.4103/0019-5154.169155; Retrieve from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4681216/
  5. Achenbach syndrome; RIGHT DIAGNOSIS; Retrieve from http://www.rightdiagnosis.com/a/achenbach_syndrome/intro.htm

4 thoughts on “Achenbach Syndrome

  • 29/10/2019 at 8:33 PM
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    As nervous as I am, I decided to finally open up about this condition because I kept it secret for too many years.
    Under the recommendation of my primary care physician, I set up an appointment with a vascular specialist. Yesterday I received a normal report back from the Vascular Doppler test. I was cleared by my Cardiologist whom I also shared my concerns with a few weeks back, having run various tests including a nuclear stress test and an halter monitor. No one can relate my symptoms to any known cause. I have a cancer history 20 years ago, so I was evaluated by my oncologist as well and could not find a connection either…it seems to be happening more frequently and it is causing me extreme anxiety. My PCP also recommended a specialist in anxiety disorders to help me cope. I have always been an active and social person but recently I am overcome with fear waiting for another episode. I only wish there was more studies done to reveal what people that suffer with this have in common. My question is whether SyndromesPedia.com is aware of any clinical trials going on?

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  • 29/10/2019 at 9:48 PM
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    I have had this condition for over 10 years. I have kept it secret and to myself as it occured infrequently perhaps once of twice a month. I didn’t think or want to believe it serious as I go regularly for blood work and nothing ever seemed a concern. However this past year after some recent surgery I have noticed it became more and more frequent in episodes. My concern level started to rise and I became more and more anxious about the next occurrence. I decided I should share this condition with my primary care Dr and my cardiologist. My primary care recommended a vascular specialist and my cardiologist ran a battery of test. The Vascular Doppler test came back normal and the cardiologist found nothing to relate back to my symptoms. Also as a past cancer patient 20 years ago, I reached out to my oncologist for an evaluation. Oncology ruled out any cancer stemming from these symptoms. My stress level has become above and beyond anything I could manage as the spontaneity of the episodes alarm me immeasurably. I have also sort out an anxiety specialist to help me cope with new fear. I have always been a social and active person but this condition has overcome me, to say the least. I was hoping to find information on clinical trials that syndromespedia.com might know of or direct me to. Thank you!

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  • 21/06/2020 at 7:49 AM
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    I notice this purple discolouration in my left big toe. I wore my running shoes with my orthotics and no socks, my shoes caused pinching on my big toe. Never in my life have I ever experienced this before. Then today June 20th, I just came back from bike riding, and I was moving my bike over to fit into a small space. I don’t recall pinching my fingers. My right hand, my index finger, located in the knuckle area. The blood vessel looked like it exploded. I had burning pain, and swelling. I went through 4 ice packs, and it s still swollen. I’m 57 yrs old. I read here where some of you have had previous conditions of other health related incidences. I only have anxiety, but other wise I am healthy. Then let’s add a new issue; I tore the meniscus/ACL and I have what is called a valgus knee (knock knee).

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  • 19/11/2020 at 1:16 AM
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    I have had this condition, undiagnosed for about 10 years. The episodes occur completely without provocation. In fact I can work hard with my hands and have nothing happen. The episodes are random in nature and interval. I have no underlying conditions except well managed high blood pressure. Each event begins with either a tiny sharp pinch or an intense itch. The area begins to swell till it is tight enough to restrict movement. Then the purple shadow shows up in the effected area. After several hours the swelling stabilizes and the discoloration starts to spread around the nearest joint till it is discolored all the way around the joint. The area will be tender, as any other bruise, for about a day. It will take from 2 – 6 days for the discoloration to fade depending on the size of the swelling and the discoloration. The best treatment I have found is to ice immediately, when the pinch or itch begins, and massage and flex as much as possible.

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