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 women^1,2.

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 acrocyanosis^1,2,3.

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 flow^1,2.

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-morbidities^1,2.

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 syndrome^1,2.

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 factors^1,2.

Pictures

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