Clinical Immunology Review Series: an Approach to the Patient with Recurrent Superﬁcial Abscesses

doi:10.1111/j.1365-2249.2008.03640.x CLINICAL IMMUNOLOGY REVIEW SERIES Clinical and Experimental Immunology Series Originator: Edward Kaminski Series Editor: Stephen Jolles Clinical Immunology Review Series: An approach to the patient with recurrent superficial abscesses ARTICLES PUBLISHED IN THIS CLINICAL IMMUNOLOGY REVIEW SERIES allergy in childhood, allergy diagnosis by use of the clinical immunology laboratory, anaphylaxis, angioedema, management of pulmonary disease in primary antibody deficiency, recurrent infections in childhood, recurrent infections in adulthood, recurrent oro-genital ulceration, recurrent superficial abscesses, urticaria, vasculitis/CTD S. L. Johnston Summary North Bristol NHS Trust, Department of Immunology and Immunogenetics, Southmead Patients may be referred to the immunology clinic for investigation of recur- Hospital, Bristol, UK rent superficial abscess formation. In the majority of adult patients this clinical presentation does not equate with an underlying primary immune deficiency. Nevertheless, recurrent mucocutaneous abscesses can be associated with significant morbidity and long-term complications, including scar- ring and fistula formation, and may be associated with underlying immune- Accepted for publication 12 February 2008 mediated disease. This review sets out an approach to the patient with Correspondence: S. L. Johnston, North Bristol recurrent superficial abscesses, focusing on the differential diagnoses, inves- NHS Trust, Department of Immunology and tigation and management of both the common causes and those associated Immunogenetics, Southmead Hospital, West- with specific immune deficiency. bury on Trym, Bristol BS10 5NB, UK. E-mail: [email protected] Keywords: abscesses, deficiency, immune, recurrent, superficial in infancy may point immediately towards a neutrophil Introduction defect such as chronic granulomatous disease (CGD); Recurrent mucocutaneous infection is a common clinical however, adult onset does not exclude a primary phagocyte problem. The majority of cases are not due to an underlying deficiency. The differential diagnoses of rash resembling primary immune deficiency. When considering immune atopic dermatitis in infancy associated with recurrent deficiency, a distinction is made between rare primary causes infection include hyper-immunoglobulin E (IgE) syndrome and the much more common secondary causes; similarly, and, in males, Wiskott–Aldrich syndrome (WAS). One of the patients presenting with recurrent superficial abscess forma- most important features of certain primary immune defi- tion can be divided into those having a specific immune ciencies is the relatively characteristic spectrum of disease- deficiency (the minority) from those with more common specific infections, which can help to focus the differential causes. The latter group includes patients with a wide spec- diagnosis. Careful attention to culture results can therefore trum of medical conditions, many being immune-mediated, guide further investigations. Phagocytic defects present encompassing staphylococcal skin carriage, chronic skin generally at a young age with susceptibility to normally disease, diabetes mellitus (DM) and inflammatory bowel non-pathogenic bacteria and fungi, in contrast to T cell disease (IBD). Iatrogenic causes of secondary immune defi- immunodeficiencies, where viral infection and candida are a ciency must be considered: for example, drug-induced particular problem. Mycobacterial infection, particularly leucopenia. Cutaneous ulceration may not be infective; addi- with low-virulence mycobacteria, may point towards a defect tional differential diagnoses include venous insufficiency in the type 1 cytokine pathway; see below. Failure to respond and pyoderma gangrenosum (PG). Psychogenic causes with as expected to anti-microbial therapy raises the possibility factitious abscess formation, particularly if the pattern of of immune deficiency. The infection site may give clues, disease is unusual, may need to be considered. for example perianal or enterocutaneous abscess formation, Where a primary immune deficiency is suspected, the dif- suggesting underlying Crohn’s disease rather than primary ferential diagnoses in paediatric and adult practice may vary, immune deficiency. and may be determined by associated clinical features. The Table 1 sets out some pointers to aid in the differential age at onset and pattern of disease, for example, can be diagnosis. When faced with the patient in clinic, the stan- discriminatory [1]. Onset of recurrent cutaneous abscesses dard format of a careful clinical history and examination © 2008 The Author(s) 397 Journal compilation © 2008 British Society for Immunology, Clinical and Experimental Immunology, 152: 397–405 CLINICAL IMMUNOLOGY REVIEW SERIES S. L. Johnston Table 1. Useful clinical features in the differential diagnosis of recurrent superficial abscesses. Clinical feature Diagnostic utility Age at onset Paediatric onset may immediately suggest phagocytic or other primary immune deficiency Underlying medical conditions Higher rates of staphylococcal carriage and increased infection risk may result - see text Family history Positive family history may suggest an inherited primary immune deficiency, the inheritance pattern, e.g. X-linked or autosomal recessive, and details of consanguinity may guide diagnosis Pattern of organ involvement The pattern of organ involvement can be useful, e.g. skin, lung, liver and bone in CGD, apocrine gland bearing skin in HS, enterocutaneous fistulae in IBD Organism Atypical mycobacteria may suggest a defect in the type 1 cytokine pathway; invasive Aspergillus infection in CGD; Pseudomonas otitis externa in DM Histology Granuloma formation in IBD, CGD and MSMD; lack of infiltrating neutrophils in LAD CGD, chronic granulomatous disease; HS, hidradenitis suppurativa; IBD, inflammatory bowel disease; MSMD, Mendelian susceptibility to mycobacterial disease; LAD, leucocyte-adhesion deficiency syndrome-1. focuses the differential diagnosis and guides appropriate Diabetes mellitus investigation. The association of recurrent infection with diabetes is well Common conditions associated with recurrent recognized, diabetes being classified by the World Health superficial abscesses Organization as a cause of secondary immune deficiency [5]. The association between diabetes and increased susceptibility to infection in general is not supported by strong evi- Staphylococcal carriage, chronic skin disease and dence; however, certain infections are more common in cutaneous trauma patients with diabetes: for example, group B Streptococcal Humans are a natural reservoir of Staphylococcus aureus. bacteraemia, tuberculosis (TB) and mucocutaneous candidi- Thirty to 50% of healthy adults are colonized, with 10–20% asis, while others occur almost exclusively in this context: being colonized persistently [2,3]. The primary site of colo- for example, rhinocerebral mucormycosis [6]. In addition, nization is the nose, with the axilla, vagina, pharynx and certain infections, e.g. TB, mucocutaneous candidiasis and damaged skin as additional sites. High colonization rates are cellulitis, may be more severe. Staphylococcal carriage rates found in patients with type 1 DM, intravenous drug users, are higher in DM. patients on haemodialysis and in surgical patients. Staphy- Diabetes impairs several aspects of host defence, the lococcal colonization is known to increase the risk of prevalence of infection correlating with glycaemic control. infection resulting from breaches in the skin or mucous Polymorphs show functional alterations in chemotaxis and membrane barrier. Leucocytes are the primary host defence phagocytosis [7], with reduced chemotaxis most marked against S. aureus and the typical pathological finding of sta- when glycaemic control is poor. The killing activity of poly- phylococcal disease is abscess formation. Patients with quan- morphs is also depressed by hyperglycaemia. The four most titative or qualitative defects in leucocyte function are important elements associated with increased infection risk therefore at increased risk of staphylococcal disease (phago- are the underlying susceptibility to infection, vascular cytic immune deficiencies are discussed below). Strains of S. disease, nerve damage and hyperglycaemia. Vascular insuffi- aureus harbouring the lukS-lukF gene encoding the Pantin– ciency and tissue hypoxia allow the growth of anaerobic Valentine leucocidin (a leucocytotoxic toxin) are frequently organisms and limit host defence mechanisms. Neuropathy associated with severe furunculosis in individuals with no known immune deficiency [4]. Table 2. Treatment of cutaneous staphylococcal carriage [4]. The combination of chronic skin disease, skin trauma A stringent 5-day decolonization regime including: and staphylococcal carriage increase the risk of abscess for- • Mupirocin nasal ointment three times daily mation; atopic eczema is a good example. Injection drug • Hand disinfection with an alcohol-based antibacterial after users are at increased risk of abscess formation because of application of the nasal ointment the increased rates of staphylococcal carriage and recurrent • Daily treatment of the skin and hair with an octenidin-based wash epithelial breaches. Although a primary immune deficiency solution is unlikely in this context, secondary immune deficiency, • Antiseptic treatment of the throat with 0·1% chlorhexidine gargle specifically human immunodeficiency virus needs to be three times daily considered and the opportunity taken

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