Background: Achenbach’s syndrome (AS) was first described by Walter Achenbach in 1958. It is a rare, benign, intermittent and self-limiting condition that causes paroxysmal bruising and pain in one or more fingers. Its pathophysiology has not yet been established, but as symptoms are typically triggered by everyday activities, local vascular fragility causing capillary micro-haemorrhages, possibly related to age or minor trauma, has been proposed. Its prevalence is unknown, although, according to a review published in 2017 by Jiménez et al, middle-aged women seem to be more commonly affected.

Aims: Presenting the case of a 19-year-old woman with AS.

Methods: A 19-year-old woman presented to the emergency department with acute painful and bruised fifth digit of the left hand. The patient reported the recurrent appearance of “blue fingers” for three months without any trauma and the situation usually improved spontaneously. On examination the finger was swollen to the level of the metacarpophalangeal joint with bruising over both surfaces of the hand, more intense in the palmar area. After clinical examination, a complete blood count and complete study of coagulation were requested.

Results: The diagnosis is essentially based on history and clinical examination, and knowledge of the condition facilitates the diagnosis. Blood counts, conventional radiology and coagulation, autoantibody and vascular flow studies are normal and not useful for the diagnosis, as shown in our case. The patient presented the last one. AS is triggered by stress and she was anxious due to admission exams. Besides that, the intermittent character of the “blue fingers” was crucial for the diagnosis of AS.

Conclusions: Due to the benign and self-limiting character of the syndrome, no specific treatment has been proposed. Patients should be counselled about the possibility of symptom recurrence in order to prevent anxiety, as the manifestations of the syndrome may be alarming.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/achenbach-syndrome-in-a-19-year-old-girl-a-clinical-case/