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PAROXYSMAL FINGER HAEMATOMAS (ACHENBACH'S SYNDROME) WITH ANGIOGRAPHIC ABNORMALITIES
PAROXYSMAL FINGER HAEMATOMAS (ACHENBACH’S SYNDROME) WITH ANGIOGRAPHIC ABNORMALITIES A. ROBERTSON, M. I. LIDDINGTON and S. P. KAY From the Department of Plastic Surgery, St. James’ University Hospital, Leeds, UK
We present two cases of women with Achenbach’s syndrome (paroxysmal finger haematomas) with abnormal angiograms. Journal of Hand Surgery (British and European Volume, 2002) 27B: 4: 391–393
biochemical parameters were normal. Angiography of the affected arm was performed 2 months after the onset of symptoms (still present) via the left femoral artery using a 5F catheter introduced into the right subclavian and brachial arteries. This revealed normal vessels to the level of the wrist and no abnormality was noted at the origins of the common digital arteries. However there was no demonstrable flow distal to the metacarpophalangeal joint of any digit despite prolonged injection with contrast (Fig 2). A small improvement in flow was observed after the administration of intra-arterial isosorbide dinitrate (1 mg), suggesting a vasospastic disorder. Spontaneous resolution followed and no further episodes were reported.
CASE REPORTS Case 1 A 41-year-old insurance company administrator presented acutely with sudden onset burning pain in the index and ring fingers of her right hand and middle finger of her left hand. Bruising subsequently appeared on the palmar aspects of the affected fingers, followed by swelling and marked restriction of movement. There had been four previous episodes. There was no history of preceding trauma and no residual discolouration or paraesthesia had occurred after each of the previous episodes. The patient was a non-smoker with minimal alcohol intake who took no medication and had an unremarkable medical history. Hand radiographs and coagulation, haematological and biochemical studies were normal. Three months after the onset of the present attack (symptoms still present), digital subtraction angiography was performed via the left femoral artery using a 5F catheter introduced into both subclavian arteries. This revealed normal vessels to the wrist level bilaterally, but significantly diminished flow to both hands suggestive of small vessel disease. The administration of intra-arterial tolazaline (25 mg) produced a clinically evident hyperaemia in the digits, but no radiographic improvement in flow. A similar episode occurred 1 month later. The patient was subsequently lost to follow-up.
DISCUSSION Achenbach’s syndrome, first described in the 1950’s, is a benign self-limiting condition which causes paroxysmal bruising in the hands or fingers (Achenbach, 1958). Women are more commonly affected than men. The mean age of the reported cases (41) is 55 (range, 30–73) years (Danilov, 1978; Deliss and Wilson, 1982;
Case 2 A 32-year-old nurse presented with a history of feeling a ‘‘pop’’ at the proximal interphalangeal joint of the index finger of her right hand. Bruising then appeared over the flexor and extensor surfaces of the finger, followed by marked swelling, pallor and paraesthesia. There was no history of preceding trauma. Several similar episodes had affected the index and middle fingers of her right hand. The patient, a non-smoker, took no medications and had an unremarkable medical history. Examination revealed finger swelling to the level of the metacarpophalangeal joint of the index finger with bruising over its flexor and extensor surfaces (Fig 1). The finger had a full range of movement and was warm and well perfused, with only a small area of diminished sensation at its tip. Hand radiographs and coagulation, haematological and
Fig 1 Bruising and swelling of the index finger (Case 2). 391
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THE JOURNAL OF HAND SURGERY VOL. 27B No. 4 AUGUST 2002
Fig 2 Angiogram demonstrating reduced flow in the digital arteries (a) before and (b) after administration of Isosorbide dinitrate (Case 2).
Eikenboom et al., 1991; Khaira et al., 2001; Layton and Cotterill, 1993; Nitzschner, 1967; Parslew and Verbov, 1995; Stieler and Heinze-Werlitz, 1990). The diagnosis is usually made on the basis of a clinical assessment. Attacks occur at variable frequencies, either spontaneously or following minor trauma. The patient commonly complains of sudden onset burning, stabbing or itching sensations in the fingers. This is followed within minutes by the appearance of bruising, usually on the palmar aspect of the fingers. The areas most commonly affected are the middle and proximal phalanges of the middle and index fingers (Eikenboom et al., 1991). The finger then becomes swollen, pale, cold and numb and movement is restricted. In the reported cases there is no consistent history of co-existing disease, although associations with acrocyanosis, gastrointestinal disorders, migraines and gall bladder disease have been noted (Layton and Cotterill, 1993). Investigation typically reveals normal coagulation and platelet counts. Histological examination of skin biopsies, when performed, have revealed changes associated with bruising or been unhelpful (Layton and Cotterill, 1993; Parslew and Verbov, 1995). Complete resolution usually occurs within a few days. Recurrent episodes occur for a variable period of time (months or years) without any apparent lasting sequelae. No specific treatment has been proposed. Previous reports have revealed no consistent radiographic or angiographic findings (Singer, 1962), and
Doppler studies have been unhelpful (Khaira et al., 2001). Although a reduced capillary resistance has been implicated, the aetiology of the syndrome is poorly understood (Nitzschner, 1967). These two cases demonstrate that there may be an association between this condition and a marked reduction in digital blood flow. This does not appear to be secondary to finger swelling, as the abnormality in the first case was at the level of the wrist. Achenbach’s syndrome must be distinguished from Painful Bruising Syndrome in which systemic features are present (Nersle and Mobacken, 1969), dermatitis artefacta, spontaneous rupture of the vincula and trauma. A similar picture may arise in Raynaud’s syndrome but a precipitant such as cold weather may be identified and this condition is usually bilateral, symmetrical and more transient. Acknowledgements Translation of German papers: Dr K. Haendlemayer.
References Achenbach W (1958). Das paroxysmale Handha¨matom. Medizinische, 52: 2138– 2140. Danilov N (1978). Contributii la sindromul W. Achenbach. Revista de Chirurgie, Oncologie Radiologie O R L Oftalmologie StomatologieChirurgie, 27: 311–312. Deliss LJ, Wilson JN (1982). Acute blue fingers in women. Journal of Bone and Joint Surgery. British Volume, 64: 458–459.
ACHENBACH’S SYNDROME Eikenboom JCJ, Cannegeiter SC, Breit E (1991). Paroxysmal Finger Haematoma: A neglected syndrome. Thrombosis and Haemostasis, 66: 266. Hersle K, Mobacken H (1969). Autoerythrocyte sensitisation syndrome (Painful bruising syndrome): report of 2 cases and review of the literature. British Journal of Dermatology, 81: 574–587. Khaira HS, Rittoo D, Smith SRG (2001). The non-ischaemic blue finger. Annals of the Royal College of Surgeons, 83: 154–157 Layton AM, Cotterill JA (1993). A case of Achenbach’s syndrome. Clinical and Experimental Dermatology, 18: 60–61. Nitzschner H (1967). Zur A¨tiologie der Fingerapoplexie. Zeitschrift fur Hautund Geschlechtskrankheiten, 42: 141–144. Parslew R, Verbov JL (1995) Achenbach syndrome [letter]. British Journal of Dermatology, 132: 319.
393 Singer R (1962). U¨ber die Symptome und die Diagnose der Fingerapoplexie (Paroxysmales Handha¨matom). Wein Klinische Wochenschrift, 74: 741–743. Stieler W, Heinze-Werlitz C (1990). Paroxysmales Fingerha¨matom. Hautarzt, 41: 270–271. Received: 31 July 2001 Accepted after revision: 16 November 2001 Mr M. I. Liddington, Department of Plastic Surgery, St James’ University Hospital Beckett Street, Leeds LS9 7TF, UK. E-mail: [email protected] r 2002 The British Society for Surgery of the Hand doi: 10.1054/jhsb.2001.0726, available online at http://www.idealibrary.com on
We present two cases of women with Achenbach’s syndrome (paroxysmal finger haematomas) with abnormal angiograms. Journal of Hand Surgery (British and European Volume, 2002) 27B: 4: 391–393
biochemical parameters were normal. Angiography of the affected arm was performed 2 months after the onset of symptoms (still present) via the left femoral artery using a 5F catheter introduced into the right subclavian and brachial arteries. This revealed normal vessels to the level of the wrist and no abnormality was noted at the origins of the common digital arteries. However there was no demonstrable flow distal to the metacarpophalangeal joint of any digit despite prolonged injection with contrast (Fig 2). A small improvement in flow was observed after the administration of intra-arterial isosorbide dinitrate (1 mg), suggesting a vasospastic disorder. Spontaneous resolution followed and no further episodes were reported.
CASE REPORTS Case 1 A 41-year-old insurance company administrator presented acutely with sudden onset burning pain in the index and ring fingers of her right hand and middle finger of her left hand. Bruising subsequently appeared on the palmar aspects of the affected fingers, followed by swelling and marked restriction of movement. There had been four previous episodes. There was no history of preceding trauma and no residual discolouration or paraesthesia had occurred after each of the previous episodes. The patient was a non-smoker with minimal alcohol intake who took no medication and had an unremarkable medical history. Hand radiographs and coagulation, haematological and biochemical studies were normal. Three months after the onset of the present attack (symptoms still present), digital subtraction angiography was performed via the left femoral artery using a 5F catheter introduced into both subclavian arteries. This revealed normal vessels to the wrist level bilaterally, but significantly diminished flow to both hands suggestive of small vessel disease. The administration of intra-arterial tolazaline (25 mg) produced a clinically evident hyperaemia in the digits, but no radiographic improvement in flow. A similar episode occurred 1 month later. The patient was subsequently lost to follow-up.
DISCUSSION Achenbach’s syndrome, first described in the 1950’s, is a benign self-limiting condition which causes paroxysmal bruising in the hands or fingers (Achenbach, 1958). Women are more commonly affected than men. The mean age of the reported cases (41) is 55 (range, 30–73) years (Danilov, 1978; Deliss and Wilson, 1982;
Case 2 A 32-year-old nurse presented with a history of feeling a ‘‘pop’’ at the proximal interphalangeal joint of the index finger of her right hand. Bruising then appeared over the flexor and extensor surfaces of the finger, followed by marked swelling, pallor and paraesthesia. There was no history of preceding trauma. Several similar episodes had affected the index and middle fingers of her right hand. The patient, a non-smoker, took no medications and had an unremarkable medical history. Examination revealed finger swelling to the level of the metacarpophalangeal joint of the index finger with bruising over its flexor and extensor surfaces (Fig 1). The finger had a full range of movement and was warm and well perfused, with only a small area of diminished sensation at its tip. Hand radiographs and coagulation, haematological and
Fig 1 Bruising and swelling of the index finger (Case 2). 391
392
THE JOURNAL OF HAND SURGERY VOL. 27B No. 4 AUGUST 2002
Fig 2 Angiogram demonstrating reduced flow in the digital arteries (a) before and (b) after administration of Isosorbide dinitrate (Case 2).
Eikenboom et al., 1991; Khaira et al., 2001; Layton and Cotterill, 1993; Nitzschner, 1967; Parslew and Verbov, 1995; Stieler and Heinze-Werlitz, 1990). The diagnosis is usually made on the basis of a clinical assessment. Attacks occur at variable frequencies, either spontaneously or following minor trauma. The patient commonly complains of sudden onset burning, stabbing or itching sensations in the fingers. This is followed within minutes by the appearance of bruising, usually on the palmar aspect of the fingers. The areas most commonly affected are the middle and proximal phalanges of the middle and index fingers (Eikenboom et al., 1991). The finger then becomes swollen, pale, cold and numb and movement is restricted. In the reported cases there is no consistent history of co-existing disease, although associations with acrocyanosis, gastrointestinal disorders, migraines and gall bladder disease have been noted (Layton and Cotterill, 1993). Investigation typically reveals normal coagulation and platelet counts. Histological examination of skin biopsies, when performed, have revealed changes associated with bruising or been unhelpful (Layton and Cotterill, 1993; Parslew and Verbov, 1995). Complete resolution usually occurs within a few days. Recurrent episodes occur for a variable period of time (months or years) without any apparent lasting sequelae. No specific treatment has been proposed. Previous reports have revealed no consistent radiographic or angiographic findings (Singer, 1962), and
Doppler studies have been unhelpful (Khaira et al., 2001). Although a reduced capillary resistance has been implicated, the aetiology of the syndrome is poorly understood (Nitzschner, 1967). These two cases demonstrate that there may be an association between this condition and a marked reduction in digital blood flow. This does not appear to be secondary to finger swelling, as the abnormality in the first case was at the level of the wrist. Achenbach’s syndrome must be distinguished from Painful Bruising Syndrome in which systemic features are present (Nersle and Mobacken, 1969), dermatitis artefacta, spontaneous rupture of the vincula and trauma. A similar picture may arise in Raynaud’s syndrome but a precipitant such as cold weather may be identified and this condition is usually bilateral, symmetrical and more transient. Acknowledgements Translation of German papers: Dr K. Haendlemayer.
References Achenbach W (1958). Das paroxysmale Handha¨matom. Medizinische, 52: 2138– 2140. Danilov N (1978). Contributii la sindromul W. Achenbach. Revista de Chirurgie, Oncologie Radiologie O R L Oftalmologie StomatologieChirurgie, 27: 311–312. Deliss LJ, Wilson JN (1982). Acute blue fingers in women. Journal of Bone and Joint Surgery. British Volume, 64: 458–459.
ACHENBACH’S SYNDROME Eikenboom JCJ, Cannegeiter SC, Breit E (1991). Paroxysmal Finger Haematoma: A neglected syndrome. Thrombosis and Haemostasis, 66: 266. Hersle K, Mobacken H (1969). Autoerythrocyte sensitisation syndrome (Painful bruising syndrome): report of 2 cases and review of the literature. British Journal of Dermatology, 81: 574–587. Khaira HS, Rittoo D, Smith SRG (2001). The non-ischaemic blue finger. Annals of the Royal College of Surgeons, 83: 154–157 Layton AM, Cotterill JA (1993). A case of Achenbach’s syndrome. Clinical and Experimental Dermatology, 18: 60–61. Nitzschner H (1967). Zur A¨tiologie der Fingerapoplexie. Zeitschrift fur Hautund Geschlechtskrankheiten, 42: 141–144. Parslew R, Verbov JL (1995) Achenbach syndrome [letter]. British Journal of Dermatology, 132: 319.
393 Singer R (1962). U¨ber die Symptome und die Diagnose der Fingerapoplexie (Paroxysmales Handha¨matom). Wein Klinische Wochenschrift, 74: 741–743. Stieler W, Heinze-Werlitz C (1990). Paroxysmales Fingerha¨matom. Hautarzt, 41: 270–271. Received: 31 July 2001 Accepted after revision: 16 November 2001 Mr M. I. Liddington, Department of Plastic Surgery, St James’ University Hospital Beckett Street, Leeds LS9 7TF, UK. E-mail: [email protected] r 2002 The British Society for Surgery of the Hand doi: 10.1054/jhsb.2001.0726, available online at http://www.idealibrary.com on