Achenbach's hand and digital paroxysmal haematomas: a possible association with joint hypermobility syndrome in two sibling cases.

Abstract

A pair of middle-aged sisters is presented who experienced episodes of spontaneous, relatively atraumatic, acute onset, painful sub-cutaneous bleeding into the digits of their hands over a number of preceding years. A literature search revealed they had Achenbach's syndrome, which is a benign and self-limiting condition that resolves completely within 2-4 days. However, a hitherto unreported association between Achenbach's syndrome and joint hypermobility with its abnormal collagen is made, along with a tentative causality hypothesis that perhaps in the presence of atypical connective tissue, the architecture and integrity of the peripheral capillary bed of the hand could be adversely affected and therefore predispose towards paroxysmal palm and/or finger bleeds.