Clinical Image of the Month: Achenbach's Syndrome
Welcome back to another Clinical Image of the Month from the case files of the Brown EM Residency.
The Case
A 30-year-old female with a history of WPW and mitral valve repair presents to the emergency department with swelling, color change, pain, and numbness of her right 4th digit for two hours. She had been outside in the cold when her finger became painful and turned blue. She reports no trauma or previous episodes. She applied ice to the finger and felt that the pain and swelling improved. No other digits were affected on both the hands and feet. She denies ever being a smoker. Family history is significant for a brother with Raynaud’s phenomenon. Her ROS is negative otherwise.
Vital signs
T 37.1°C (98.7°F), BP 120/77, HR 80, RR 16, SpO2 100% on RA
Exam
Constitutional: She is oriented to person, place, and time. She appears well-developed and well-nourished. No distress.
HEENT:
Head: Normocephalic and atraumatic.
Eyes: EOM are normal.
Neck: Normal range of motion.
Cardiovascular: Regular rate and rhythm. Intact distal pulses.
Pulmonary/Chest: No respiratory distress.
Muskuloskeletal: Normal range of motion. She exhibits edema (4th finger right). She exhibits no tenderness or deformity.
Neurological: She exhibits normal muscle tone.
Skin: She is not diaphoretic. Ecchymosis of R 4th finger, spares finger tip. Fingertip is pink with normal capillary refill.
Psychiatric: She has a normal mood and affect.
Images
What’s the (differential) diagnosis?
Raynaud’s phenomenon
Traumatic hematoma
Fracture/dislocation
Arterial thrombosis/embolus
Vasculitis
Vasospasm
Acrocyanosis
Something you’ve never heard of
The disease
Achenbach’s syndrome, or paroxysmal finger hematoma, is a rare, benign, self-limiting condition of spontaneous hemorrhage in the hands and fingers. It was first described by a physician named Walter Achenbach in 1955. It is usually described by patients as an abrupt onset of burning pain in one or more digits with associated symptoms including swelling, cyanosis, pallor, coldness, numbness, itching, and difficulty moving the affected digits. Coloration can range from pallor to blue, and typically affects the palmar aspect of the involved digit around the base or MCP joint. It can also be entirely painless. The index finger is most commonly involved, follow by the middle finger. Rare cases may involve the thumb. For unknown reasons AS more commonly affects the left hand. Diagnosis is made clinically and testing will be normal, including hematologic tests, coagulation studies, imaging, and vascular flow studies. Biopsy may show nonspecific signs including minor bleeding and inflammation. Symptoms will improve on their own but may take days to weeks to fully resolve, and it will often relapse. Middle-aged women are the most common patients who present with this rare syndrome.
Management
Achenbach’s syndrome is a diagnosis of exclusion, and therefore organic causes of pain and hemorrhage or bruising in a digit should first be excluded. Underlying coagulopathy, vascular insufficiency, and autoimmune causes are all within the differential and should be excluded when there is high enough suspicion. There are no consistent angiographic or radiographic findings, and there are no clear disease associations. Etiology may possibly be related to vascular fragility as occasional cases occur after minor trauma, however there is no evidence to support this definitively. Once a diagnosis is made by excluding other possible causes patients with this rare condition should be reassured of its benign nature. They should also be counseled about the likelihood of recurrence to prevent anxiety if they develop symptoms again.
Our patient’s presentation was atypical in that she was younger than the usual presenting age and the 4th digit isn’t commonly affected in an isolated manner. The actual color changes were typical of AS in that they involved the more proximal part of the digit. For this patient, it was important to rule out a vascular cause of her symptoms given that she had a history of a dysrhythmogenic condition and a valve repair, both of which could predispose her to cardioembolic events resulting in arterial occlusion. Clinically her finger did not appear ischemic, and her cardiac exam was normal making this unlikely to be the case.
Attempts to rewarm the finger further did not result in color change back to normal. Given the normal appearance and perfusion of the distal finger there was no concern for a vascular emergency. An x-ray of the finger was normal and ruled out fracture. The patient was provided with reassurance and sent home with return precautions and PCP follow-up.
Reviewers: Dr. Gita Pensa and Dr. Kelly Wong
References
Ahmed Z et al. Painful Blue Finger – Achenbach’s Syndrome: Two Case Reports. EJVES Short Reports. 2018; 40:1-2.
Frerix M, Richter K, Müller-Ladner U, Hermann W. Achenbach’s syndrome (paroxysmal finger hematoma) with capillaroscopic evidence of microhemorrhages. Arthritis Rheumatol. 2015; 67:1073. DOI: 10.1002/art.39003.
Huikeshoven M, de Priester JA, Engel AF. A case of spontaneous wrist haematoma in Achenbach syndrome. Journal of Hand Surgery (European Volume). 2009; 34, 4:551-552. DOI: 10.1177/1753193409103731
Jiménez PR et al. Achenbach’s syndrome: Case report and systematic review of the literature. Rev Colomb Reumatol. 2017; 24:230–236.
Robertson A, Liddington MI, Kay SP. Paroxysmal finger haematomas (Achenbach’s syndrome) with angiographic abnormalities. Journal of Hand Surgery. 2002; 27:391-3. DOI: 10.1054/jhsb.2001.0726
Takeuchi H, Uchida HA, Okuyama Y, et al. Acutely idiopathic blue fingers: a young man with Achenbach’s Syndrome. BMJ Case Rep. 2016. DOI:10.1136/bcr-2016-214491.
Watchcorn RE et al. Paroxysmal purple palmar macules with a rare aetiology. Clinical and Experimental Dermatology. 2017; 42:561–563. doi: 10.1111/ced.13101.
Yamamoto Y, Yamamoto S. Achenbach’s syndrome. New England Journal of Medicine. 2017; 376: 26. DOI: 10.1056/NEJMicm1610146.