An Approach to Investigation of Easy Bruising

488 Arch Dis Child 2001;84:488–491 PERSONAL PRACTICE Arch Dis Child: first published as 10.1136/adc.84.6.488 on 1 June 2001. Downloaded from An approach to investigation of easy bruising A Vora, M Makris Easy bruising presents several investigative its absence excludes significant abnormality. dilemmas in primary and secondary practice, Steroid inhaler or non-steroidal anti- not least because it raises the spectre of physi- inflammatory drug (NSAID) use, current or cal abuse. When, and to what extent, should previous, should be excluded in those cases general paediatricians investigate before refer- where an abnormal pattern of bruising is the ral to a specialist? When can it be safely sole cause for concern. Long term use of high concluded that an underlying bleeding disor- dose inhaled steroids, and even a single dose of der has been excluded, so that further investi- NSAIDs, can predispose to easy bruising that gations can focus on ruling out suspected non- persists for weeks to months after cessation. accidental injury? When, despite an abnormal Other drugs, such as anticonvulsants, may also clotting test, should the latter investigations be a cause of easy bruising and a comprehen- still be pursued? sive drug history should be an important part The intention of this review is to attempt to of the initial assessment. answer these questions from the perspective of The target of further investigation should routine practice without recourse to a compre- therefore be patients with bruising over the hensive review of bleeding disorders or non- trunk, neck, or face, irrespective of limb or accidental injury. We intend to address only the mucosal bleeding, those with excessive blood patient seen electively in the outpatient clinic loss after minor surgery, or a positive family for investigation of easy bruising, not an acutely history. ill child presenting to accident and emergency with purpura, whose diVerential diagnosis varies from, most often, no cause found, to, less Causes of abnormal bruising commonly, meningococcal sepsis or Henoch– Abnormal bruising is not exclusively a result of Schonlein purpura. haemostatic disorders.4 In addition to non- accidental injury, collagen disorders, though http://adc.bmj.com/ rare, should be considered in the diVerential Distinguishing “normal” from 5 “abnormal” bruising diagnoses. Bruising caused by accidental injury is common around the age of 1, when most infants THROMBOCYTOPENIA have started “cruising”.1 To distinguish “ab- Immune thrombocytopenic purpura (ITP) is normal” from “normal” bruising requires the commonest haemostatic disorder of child- attention to the pattern of bruising, associated hood to present with easy bruising, usually on September 27, 2021 by guest. Protected copyright. symptoms, and drug and family history.2 As a associated with petechiae, purpura, and mu- rule, normal bruising is restricted to the lower cosal bleeding. The diagnosis is of exclusion, limbs,1 not associated with petechiae, purpura, and made on the basis of an otherwise well or mucosal bleeding, and the family history is patient, without lymphadenopathy or organo- negative. The latter should be interpreted cau- megaly, with isolated thrombocytopenia and a tiously as it is often negative in disorders of normal blood film and clotting screen. The lat- haemostasis, and not infrequently positive in ter two in a well child excludes leukaemia, families with unexplained easy bruising or meningococcal septicaemia, and haemolytic epistaxis, who may carry the nebulous entity of uraemic syndrome. Persistence of thrombocy- “increased skin fragility” which is usually asso- topenia beyond a few months from presenta- ciated with “hyperextensible” joints, but with- tion should trigger referral to a specialist for out the classic features of Ehlers–Danlos further investigations. These should be di- syndrome (see below).3 It is also important to rected towards exclusion of rare congenital obtain a comprehensive family history, which causes of isolated thrombocytopenia (such as SheYeld Children’s includes grandparents and the extended fam- May–Hegglin syndrome and other giant plate- Hospital, SheYeld ily, and taking special care to note, where this let familial thrombocytopenia syndromes, Fan- S10 2TH, UK may be the case, the diVerent parentage of all coni’s anaemia, and amegakaryocytic thrombo- A Vora the siblings. cytopenia), before concluding that the patient M Makris Documenting a history of the response to has chronic ITP.Although investigations at this Correspondence to: haemostatic stress is useful in determining the stage may include examination of the bone Dr Vora likelihood of a significant bleeding disorder. marrow, if this has not already been performed, ajay.vora@ Excessive bleeding after tooth extractions or careful reexamination of the blood film and sheVch-tr.trent.nhs.uk minor surgery (for example, tonsillectomy) is a parental blood counts may be the simplest way Accepted 31 January 2001 characteristic of disorders of haemostasis, and to exclude some of these rare conditions. www.archdischild.com An approach to investigation of easy bruising 489 PLATELET FUNCTION DISORDERS Congenital deficiencies of other coagulation Poor platelet aggregability is caused by a factors are much rarer and variably associated Arch Dis Child: first published as 10.1136/adc.84.6.488 on 1 June 2001. Downloaded from variety of acquired and inherited disorders, the with a risk of bleeding, except for factor XII commonest being NSAID use.6 Inherited deficiency, which is always asymptomatic. disorders of platelet function are rare, but should be suspected in a patient with symp- Acquired toms of thrombocytopenia but a normal plate- Sick neonates bruise easily, usually as a result let count, or mild thrombocytopenia relative to of a low platelet count. Bruising in infants the severity of haemorrhagic symptoms. The younger than 9 months is rare, and should best known, and easiest to diagnose, are always prompt a search for a cause.1 Physical Glanzmann’s thrombasthenia and Bernard abuse should be suspected at this age, more Soulier syndrome, which result from a measur- than any other, if another explanation for able (by flow cytometry) lack of expression of bruising is not found. platelet membrane receptors essential for acti- An uncommon cause of bruising at this age vation and aggregation. Storage pool deficiency is haemorrhagic disease of the newborn is harder to diagnose, as few patients have the (HDN), which occurs when prophylactic vita- classical clinical and laboratory features, and min K has not been administered at birth, or the laboratory tests required to exclude it are has been given orally to subsequently breast fed diYcult to perform and interpret. Inheritance infants without the one and three month of most platelet function disorders is autosomal boosters. Unrecognised, it can result in cata- recessive, so it may not be apparent without strophic intracranial haemorrhage. Once rec- testing the extended family. Given the diYcul- ognised it is easily treated with intravenous ties in diagnosis, it is probably best to refer vitamin K. patients suspected of a platelet function defect Outside infancy, vitamin K deficiency is to a specialist. usually caused by malabsorption, liver disease, or a combination of the two. Coeliac disease DISORDERS OF COAGULATION FACTORS may present with easy bruising as the sole Inherited symptom, although most patients will have Autosomal dominantly inherited Von Wille- other signs of malabsorption. Similarly, inflam- brand’s disease (VWD) is the commonest con- matory bowel disease and chronic liver disease genital disorder of haemostasis, aVecting up to may have easy bruising as a dominant present- 1% of the population7; it often presents with ing symptom. These diagnoses should be clini- easy bruising as the sole symptom, although cally obvious, but are worth excluding as a mucosal bleeding is also common. Purpura and cause of isolated prolongation of the pro- petechiae are not common despite the abnor- thrombin time. mal platelet aggregation, which is a feature of this condition along with a variable reduction COLLAGEN DISORDERS http://adc.bmj.com/ in concentrations of von Willebrand factor Vascular integrity is essential for primary (VWF) and factor VIII. Post-pubertal females haemostasis to be eVective. Defective collagen may have menorrhagia. Family history is compromises capillary and skin elasticity, frequently positive, but may be silent and only thereby manifesting symptoms similar to those uncovered on parental testing. The majority of of thrombocytopenia or platelet function de- cases are mild with concentrations just below fect.8 Not all patients have the classic features the normal range. This may cause diagnostic of Ehler–Danlos syndrome,3 Marfan’s syn- diYculty, as the venepuncture ordeal can drome, or acquired autoimmune disorders. A on September 27, 2021 by guest. Protected copyright. stimulate release of factor VIII and VWF from simple test of thumb hyperflexibility is claimed endothelial stores, often pushing marginally to identify patients with a mild inherited bleed- sub-normal concentrations to within the nor- ing diathesis without abnormalities of haemos- mal range. Where suspicion is strong, and con- tasis or other features of a collagen disorder.9 centrations borderline normal, repeat tests may be justified, but should be postponed until a NON-ACCIDENTAL INJURY management decision rests on the diagnosis Suspicion of this comes from a variety of medi- (for example, impending

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