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A Painful Blue Thumb: A Case of Achenbach's Syndrome

      Abstract

      Achenbach syndrome is a rare, self-limiting condition, which causes paroxysmal bruising in the hand or fingers. Etiology is still unknown. The acute phase can be very alarming, and could suggest a more serious vascular disease. Clinicians should be aware of the existence of this condition and not undertake needless invasive investigations.

      Keywords

      1. Introduction

      Achenbach's syndrome is a rare clinical entity consisting of spontaneous paroxysmal bruising on the volar aspects of fingers or hand associated with burning pain and swelling of the digits. It is important to be aware of this syndrome as the symptoms these patients present could suggest a more serious vascular disease and lead to invasive investigations.

      2. Case Report

      A 60-year-old non-smoker woman attended our emergency unit with a history of sudden onset of pain in the distal phalange of her thumb. Subsequently bruising appeared on the volar aspect of the pulp accompanied by marked pain and coldness (Fig. 1). She had a previous similar episode, which resolved completely and spontaneously within a few days. There was no history of trauma.
      The patient occasionally took medications against migraine but had not done so for the last 3 months. She had otherwise an unremarkable medical history. Examination revealed thumb swelling at the level of the distal phalange with bruising over its volar surface. The thumb was well perfused and showed an unrestricted mobility. Blood parameters including coagulation tests and radiographs were normal. No consistent findings were noted at a high resolution sonography. Colour Doppler investigation and measurement of finger-tip pressure revealed unaltered perfusion flow. No specific treatment was proposed and spontaneous resolution followed within 2 weeks.

      3. Discussion

      Achenbach's syndrome was first described in the 1950s as a rare, benign self-limiting condition which causes paroxysmal bruising in the hand or fingers.
      • Achenbach W.
      Das paroxysmale Handhämatom.
      Women are more commonly affected than men and the age of onset is predominantly over 50. The phalanges of the middle and index finger are the most commonly affected.
      • Eikenboom J.C.J.
      • Cannegeiter S.C.
      • Breit E.
      Paroxysmal finger haematoma: A neglected syndrome.
      Patients report a spontaneous sudden onset of burning or itching sensations in the finger, followed within minutes by the appearance of bruising, usually on the palmar aspect of the fingers.
      • Eikenboom J.C.J.
      • Cannegeiter S.C.
      • Breit E.
      Paroxysmal finger haematoma: A neglected syndrome.
      • Zimmerli L.
      • Dobler C.
      Aschwanden. Recurrent paroxysmal finger hematoma (Achenbach syndrome).
      The finger then becomes swollen, pale and cold. Movement can be restricted. The recurrence rate is very variable and the haematomas generally resorb within a few days. Associations with acrocyanosis, gastrointestinal disorders, migraines and gall bladder disease have been noted.
      • Layton A.M.
      • Cotterill J.A.
      A case of Achenbach's syndrome.
      The diagnosis is made on the basis of the clinical assessment, because coagulation, haematological and biochemical parameters, as well conventional radiology and Doppler examination are normal.
      • Khaira H.S.
      • Rittoo D.
      • Smith S.R.G.
      The non-ischaemic blue finger.
      These authors also reported that the distribution of the discoloration is limited on the volar aspect of the digit but usually does not involve the tip.
      The aetiology is not yet established but is probably of vascular origin. In a recent publication of two cases of women with Achenbach's syndrome authors suggest that there may be an association with a marked reduction in digital blood flow at arteriography.
      • Robertson A.
      • Liddington M.I.
      • Kay S.P.
      Paroxysmal finger haematomas (Achenbach's syndrome) with angiographic abnormalities.
      The role of anti-migraine medication (ergotamine) may influence the microvascular digital flow and induce a vasospastic reaction.
      The clinical presentation of the syndrome must be distinguished from other conditions such as painful bruising syndrome (autoerythrocyte sensitisation syndrome, which commonly involves the legs and trunk) dermatitis artefacta, spontaneous rupture of the vincula, trauma and Raynaud's syndrome.
      As the acute phase of the syndrome can be very alarming to both the patient and the physician, they should both be aware of the existence and benignity of this condition and not undergo unnecessary invasive investigations.

      References

        • Achenbach W.
        Das paroxysmale Handhämatom.
        Medizinische. 1958; 52: 2138-2140
        • Eikenboom J.C.J.
        • Cannegeiter S.C.
        • Breit E.
        Paroxysmal finger haematoma: A neglected syndrome.
        Thromb Haemost. 1991; 66: 266
        • Zimmerli L.
        • Dobler C.
        Aschwanden. Recurrent paroxysmal finger hematoma (Achenbach syndrome).
        Schweiz Rundsch Med Prax. 2002; 91: 1664-1666
        • Layton A.M.
        • Cotterill J.A.
        A case of Achenbach's syndrome.
        Clin Exp Dermatol. 1993; 18: 60-61
        • Khaira H.S.
        • Rittoo D.
        • Smith S.R.G.
        The non-ischaemic blue finger.
        Ann R Coll Surg. 2001; 83: 154-157
        • Robertson A.
        • Liddington M.I.
        • Kay S.P.
        Paroxysmal finger haematomas (Achenbach's syndrome) with angiographic abnormalities.
        J Hand Surg [Br]. 2002; 27: 391-393

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