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Putting a finger on the diagnosis of Achenbach syndrome


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By Warren R. Heymann, MD, FAAD
July 28, 2021
Vol. 3, No. 30

Please study the image below first. Castillo et al reported the case a 48-year-old woman complaining of acute pain, swelling, and ecchymosis of the volar aspect of her right third finger, sparing the fingertip, when walking her dog. (1) Be honest — would you have diagnosed Achenbach syndrome (AS)? Not I. As I remind my residents, it is challenging to render a diagnosis that you have never heard of. After reading this commentary, those unfamiliar with this diagnosis should be able to recognize it, differentiating from other diseases presenting similarly.

The disorder was first described in 1958 by the German physician Walter Achenbach, who presented 6 cases of women with recurrent episodes of acute pain on the palmar aspect of one or more digits followed by blue discoloration. The syndrome is also known as paroxysmal finger hematoma (PFH) or blue finger syndrome. According to Godoy and Tabares, “Prodromal symptoms, such as pain, tingling, and itching, may occur from minutes to hours before the color change appears; however, the subdermal bleeding usually stops spontaneously or after local pressure is applied, and the coloration changes usually disappear within 3–6 days. Interestingly, the lesions usually disappear without going through the typical stages of ecchymosis resorption. Moreover, the distal phalanges and the nail bed are not involved, in contrast to what has been observed previously in ischemic finger diseases. Overall, the course of this condition is benign, and the symptoms usually resolve without permanent sequelae.” (2) Kordzadeh et al, in their systematic review of the literature note that AS is a predominantly observed in women with a median age of 49.5 years with a median time to resolution of symptoms (without any intervention) of 4 days. (3) In an analysis of 24 patients, AS was most commonly seen on the right index finger of middle-aged female patients. (4)

Image for DWII on Achenbach syndrome
Image for DWII on Achenbach syndrome
Image of the patient from reference 1, with permission, courtesy of Andrew E. Werchniak, MD.

The etiology of AS is unknown. Most cases are unassociated with trauma, and if trauma is implicated, it is very minor, such as from a door handle or opening a jar. Genetic factors may be relevant as in the personal remembrance of Cohen, who describes his case and his mother’s. (5) There are no consistent associations, although there are reports linking rheumatoid arthritis (6), migraines, acrocyanosis, and gastrointestinal disorders. (1) Laboratory evaluations, including CBC, coagulation studies, rheumatological serologies, ultrasonography, and capillaroscopy are unremarkable. Other than hemorrhage, skin biopsies are unrevealing. (4) Although some arteriographic observations have revealed that digital blood flow is significantly reduced in these patients, the presumption is that bleeding is due to venous rather than arterial hemorrhaging. (2)

In the differential diagnosis, the most immediate concern is acute limb ischemia (due to thrombosis or embolization) — in that circumstance, lesions tend to be distal, associated with a cold peripheral temperature, and potential infarction. Buerger’s disease (thromboangiitis obliterans) is observed in smokers and demonstrates distal ulceration. Raynaud syndrome is more chronic, displaying a panoply of color changes, and is not limited to the volar aspect of the fingers. Patients with acrocyanosis present with symmetrical involvement of the hands fingers, and face. Pernio (chilblains) is usually seen on the toes in cold weather. (3,7) “COVID toes” may be a consideration. Pavlovic and Loubser suggest that the term “paroxysmal acral hematoma” may be more appropriate after observing a 51-year-old with AS involving the palm, finger, and second toe. (8) Finally, the Gardner-Diamond syndrome (psychogenic purpura) — an entity that I have never understood — enters the differential diagnosis, presenting with more generalized ecchymotic lesions. (9)

Regarding AS, Yamamoto and Yamamoto state: “Patients may seek medical care out of fear because of the sudden pain and bleeding; therefore, diagnosing this condition may relieve anxiety.” (10) If considering AS (especially for the first episode), I still think that it would be reasonable to check some rudimentary laboratory studies (CBC, ESR, PT/PTT, ANA, and arterial and venous Doppler ultrasonagraphy). With normal studies and resolution in a matter of days, the diagnosis of AS can be confirmed. Because of its self-limited nature, other than analgesia, reassurance of the benign nature of the condition should alleviate patient concerns.

I am most fortunate to work with brilliant colleagues. When I showed the image (without any history) to Dr. Justin Green, I asked him to offer a diagnosis — his immediate response was “Achenbach Syndrome.” I was taken aback and asked how he knew that. “Because I have it!” Dr. Green will offer “Our Expert’s Viewpoint” from the vantage point of a physician who has experienced AS.

Point to Remember: Achenbach syndrome (paroxysmal finger hematoma) is a benign entity with an alarming presentation of pain, swelling, and ecchymosis. Overzealous evaluation may be avoided after a careful consideration of the differential diagnosis.

Our expert’s viewpoint

Justin J. Green, MD
Assistant Professor of Medicine (Dermatology)
Cooper Medical School of Rowan Universit
y

“I wish somebody would have told me babe
Some day, these will be the good old days” - Macklemore and Kesha

I am certain that Macklemore and Kesha were not reminiscing about reading a weighty hard cover medical textbook in their catchy pop song “Good Old Days.”

There was a time in the not-too-distant past that all of our medical knowledge arrived a little bit slower. We would request journal articles from the library and purchase textbooks from a medical bookstore. During my residency, our cohesive group of three would read dermatology texts cover to cover and quiz each other on the readings over a cup of coffee. It was then, while reading Andrews’ Diseases of the Skin, 9th Edition that I first encountered Achenbach’s Syndrome. There was just one small paragraph at the bottom of page 1028 describing this entity. Tucked away in the chapter on Cutaneous Vascular Diseases and sharing a page with Gardner-Diamond Syndrome, “paroxysmal hand hematoma” did not seem too relevant to my upcoming career and board examination.

Several years advanced and I was a junior attending, rounding one night in the NICU. After scrubbing my hands vigorously I experienced a sudden pain in my left 4th finger. Within minutes I watched as a purple-blue hue appeared and expanded over the volar, lateral aspect of my finger between the PIP and DIP joints. It was exquisitely tender and slightly edematous. The purpura lasted a few days, but the pain and tenderness subsided rather quickly. I showed my resident physician and proclaimed “This must be Achenbach’s syndrome.” It appeared she had not yet read Andrews’.

In my 18 years of practice, I have only diagnosed this condition three more times. One time I diagnosed my mother with her iPhone texted image. It also has recurred on my same finger — just once. Each of the other cases I have diagnosed involved middle-aged women who gave a history of Raynaud’s phenomenon — an association reported by Carpentier et al. (11)

I suspect paroxysmal finger hematoma is much more common than we think. After all, the clinical findings are fleeting, and most dermatology appointments are limited for urgent issues. Telehealth accessibility and the public’s concern for COVID fingers and toes might lead to the dermatology community encountering this more often.

Since my residency 20 years ago a lot has changed. Gone is the 90-hour week. Epocrates app on an iPhone has replaced the voluminous, microscopically written print of the PDR. Injectable IL-17 and IL-23 inhibitors have supplanted oral cyclosporine for the treatment of severe psoriasis. PD-1 inhibitors are used instead of dacarbazine for metastatic melanoma. Electronic medical records have replaced the indecipherable script of Dr. Heymann’s written notes.

When I evaluate the advances in our field over the past two decades it is clear that medical students, dermatologists, and our patients are much better off now than ever before. However, part of me wonders whether I would have ever come across Achenbach’s syndrome but for the presence of a good old textbook.

“’Cause someday soon, your whole life’s gonna change
You’ll miss the magic of the good old days” - Macklemore and Kesha 2017

  1. Castillo SA, Dinulos JG, Werchniak AE. Painful ecchymosis of the finger: A case of Achenbach syndrome. Int J Dermatol 2020; 59: 862-863.

  2. Godoy A, Tabares AH. Achenbach syndrome (paroxysmal finger hematoma). Vasc Med 2019; 24: 361-366.

  3. Kordzadeh A, Caine PL, Jonas A, Rhodes KM, Panayiotopoulos YP. Is Achenbach syndrome a surgical emergency? A systematic review. Eur J Trauma Emerg Surg 2016; 42: 439-443.

  4. Ada F, Kasimzade F. Analysis of 24 patients with Achenbach syndrome. World J Clin Cases 2019; 7: 1103-1110.

  5. Cohen SN. Personal observations of Achenbach syndrome. Clin Exp Dermatol 2018; 43: 933-934.

  6. Manappallil RG, Jayraj J. Blue finger syndrome: An unusual presentation of rheumatoid arthritis. J Clin Diagn Res 2017; 11(5): OD06–OD07.

  7. Yamada T. Achenbach’s syndrome in an elderly woman. J Gen Fam Med 2018; 19: 65-66.

  8. Pavlovic MD, Loubser MD. Paroxysmal acral haematoma is a more appropriate name for Achenbach syndrome. Clin Exp Dermatol 2019; 44: e18-e19.

  9. Sridharan M, Ali U, Hook C, Nichols WL, Pruthi RK. The Mayo Clinic experience with psychogenic purpura. Am J Med Sci 2019; 357: 411-420.

  10. Yamamoto Y, Yamamoto S. Achenbach’s syndrome. N Engl J Med 2017; 376: 26.

  11. Carpentier PH, Maricq HR, Biro C, Jiguet M, Seinturier C. Paroxysmal finger haematoma--a benign acrosyndrome occurring in middle-aged women. Vasa. 2016 Jan;45(1):57-62. doi: 10.1024/0301-1526/a000496.



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