6 Infections and inflammatory conditions

UTI is currently defined as the inflammatory response of the urothelium to bacterial invasion. This inflammatory response causes a constellation of symptoms. Bladder infection (cystitis) causes frequent small volume voids, urgency, suprapubic pain or discomfort, and urethral ‘burning’ on voiding (dysuria). Acute kidney infection (acute pyelonephritis) causes symptoms of fever, chills, malaise, and loin pain, often with associated LUTS of frequency, urgency, and urethral pain on voiding. The strict requirement for >10⁵ bacteria/mL of MSU specimen is no longer required to make a diagnosis of UTI. In symptomatic patients, many clinicians will now make a diagnosis of UTI with bacterial counts of >10²/mL. Current recommendations for diagnosing UTI from MSU culture is shown in Table 6.2.

Bacteriuria: is the presence of bacteria in the urine. Bacteriuria may be asymptomatic or symptomatic. Bacteriuria without pyuria indicates the presence of bacterial colonization of the urine rather than the presence of active infection.

Pyuria: is the presence of white blood cells in the urine (implying an inflammatory response of the urothelium to bacterial infection or in the absence of bacteriuria (sterile pyuria), some other pathology such as carcinoma in situ, TB infection, bladder stones, or other inflammatory conditions.

An uncomplicated UTI: is one occurring in a patient with a structurally and functionally normal urinary tract. The majority of such patients are women who respond quickly to a short course of antibiotics.

A complicated UTI: is one occurring in the presence of an underlying anatomical or functional abnormality (e.g. incomplete bladder emptying secondary to BOO or DSD in SCI), renal or bladder stones, colovesical fistula, etc. Other factors suggesting a potential complicated UTI are diabetes mellitus, immunosuppression, hospital-acquired infection, indwelling catheter, recent urinary tract intervention, and a failure of response to appropriate treatment. Most UTIs in men occur in association with a structural or functional abnormality and are, therefore, defined as complicated UTIs. Complicated UTIs take longer to respond to antibiotic treatment than uncomplicated UTIs and if there is an untreated underlying abnormality, they will usually recur within days, weeks, or months.

UTIs may be isolated, recurrent, or unresolved.

Most UTIs are caused by faecal-derived bacteria that are facultative anaerobes (i.e. they can grow under both anaerobic and non-anaerobic conditions) (see Table 6.3).

Infection in a subject with a normal functional and anatomical urinary tract. Most UTIs are bacterial in origin. The most common cause is Escherichia coli (E. coli), a Gram-negative bacillus, which accounts for 85% of community-acquired and 50% of hospital-acquired infections. Other common causative organisms include Staphylococcus saprophyticus, Proteus mirabilis, and Klebsiella.

Infection in a subject with a functional or anatomical abnormality of the urinary tract, underlying risk factors, or failure to respond to therapy. E. coli is responsible for up to 50% of cases. Other causes include Enterococci, Staphylococci, Pseudomonas, Proteus, Klebsiella, and other enterobacteria.

The vast majority of UTIs result from infection ascending retrogradely up the urethra. Bacteria, derived from the large bowel, colonize the perineum, vagina, and distal urethra. They ascend along the urethra to the bladder (increased risk in females as urethra shorter), causing cystitis. From the bladder, they may ascend via the ureters to involve the kidneys (pyelonephritis). Reflux is not necessary for infection to ascend to the kidneys, but it will encourage ascending infection as will any process that impairs ureteric peristalsis (e.g. ureteric obstruction, Gram-negative organisms and endotoxins, pregnancy). Infection that ascends to involve the kidneys is also more likely where the infecting organism has P pili (filamentous protein appendages, also known as fimbriae, which allow binding of bacteria to the surface of epithelial cells).

Haematogenous: uncommon, but is seen with Staphylococcus (S.) aureus, Candida fungaemia, and Mycobacterium (M.) tuberculosis (causing TB).

Infection via lymphatics: seen rarely in inflammatory bowel disease and from retroperitoneal abscess.

Many Gram-negative bacteria have pili (also known as fimbriae) on their cell surface, which aid attachment to urothelial cells of the host. A typical piliated cell may contain 100–400 pili. Pili are 5–10nm in diameter and up to 2μm long. E. coli produces a number of antigenically and functionally different types of pili on the same cell; other strains may produce only a single type and in some isolates, no pili are seen (such as Dr adhesin associated with UTI in pregnant women and children). Pili are defined functionally by their ability to mediate haemagglutination (clumping of red blood cells) of specific types of erythrocytes. Mannose-sensitive (type 1) pili are produced by all strains of E. coli and are associated with cystitis. Certain pathogenic types of E. coli also produce mannose-resistant P pili and are associated with pyelonephritis. S pili are associated with infection of both the bladder and kidneys.

Host defences: factors that protect against UTI include the following.

Cystitis: is infection and/or inflammation of the bladder.

Presentation: frequent voiding of small volumes, dysuria, urgency, offensive urine, suprapubic pain, haematuria, fever ± incontinence.

Leukocyte esterase activity detects the presence of white blood cells (WBC) in the urine. Leukocyte esterase is produced by neutrophils and causes a colour change in a chromogen salt on the dipstick. Not all patients with bacteriuria have significant pyuria (sensitivity of 75–95% for detection of infection, i.e. 5–25% of patients with infection will have a negative leukocyte esterase test, erroneously suggesting that they have no infection).

Remember, there are many causes for pyuria (and, therefore, a positive leukocyte esterase test occurring in the absence of bacteria on urine microscopy). This is so-called sterile pyuria and it occurs with TB infection, renal calculi, bladder calculi, glomerulonephritis, interstitial cystitis, and carcinoma in situ. Thus, the leukocyte esterase dipstick test may be truly positive in the absence of infection.

Nitrites are not normally found in urine and their presence suggests the possibility of bacteriuria. Many species of Gram-negative bacteria can convert nitrates to nitrites and these are detected in the urine by a reaction with the reagents on the dipstick which form a red azo dye. The specificity of the nitrite dipstick for detecting bacteriuria is >90% (false positive nitrite testing can occur with contamination). The sensitivity is 35–85% (i.e. false negatives are common—a negative dipstick in the presence of active infection) and is less accurate in urine containing <10⁵ organisms/mL. Hence, if the nitrite dipstick test is positive, the patient probably has a UTI, but a negative test often occurs in the presence of infection.

Cloudy urine, which is positive for WBCs on dipstick and is nitrite-positive, is very likely to be infected.

Haemoglobin has a peroxidase-like activity, causing oxidation of a chromogen indicator on the dipstick, which changes colour when oxidized. False positives are seen with menstrual blood and dehydration.

Urinary pH usually lies between 5.5 and 6.5 (range 4.5–8). A persistent alkaline pH associated with UTI indicates a risk of stones. Urease-producing bacteria (such as Proteus mirabilis) hydrolyze urea to ammonia and carbon dioxide, leading to the formation of magnesium, calcium, ammonium phosphate stones (triple phosphate or struvite calculi).

If the urine specimen contains large numbers of squamous epithelial cells (cells which are derived from the foreskin, vaginal, or distal urethral epithelium), this suggests contamination of the specimen and the presence of bacteria in this situation may indicate a false positive result. The finding of pyuria and red blood cells suggests the presence of active infection.

Determined by the clinical scenario. If this is a one-off infection in an otherwise healthy individual, no further investigations are required. However, further investigations are required if:

These further investigations will include a KUB X-ray ± IVU (looking for infection stones in the kidney; avoid in pregnant women), renal USS ± cystoscopy.

Symptoms of cystitis can also be caused by:

The aim is to eliminate bacterial growth from the urine. Empirical treatment involves the administration of antibiotics according to the clinical presentation and most likely causative organism before culture sensitivities are available (Table 6.4). Men are often affected by complicated UTI and may require longer treatments as may patients with uncorrectable structural or functional abnormalities (e.g. indwelling catheters, neuropathic bladders).

Organisms susceptible to concentrations of an antibiotic in the urine (or serum) after the recommended clinical dosing are termed ‘sensitive’ and those that do not respond are ‘resistant’. Bacterial resistance may be intrinsic (e.g. Proteus is intrinsically resistant to nitrofurantoin) via selection of a resistant mutant during initial treatment or genetically transferred between bacteria by R plasmids. Antibiotic-resistant organisms that cause complicated UTI include Gram-negative bacteria that produce AmpC enzymes or extended spectrum B-lactamases (ESBLs) (which are often multidrug resistant) and Gram-positive cocci such as methicillin-resistant Staphylococcus aureus (MRSA), methicillin-resistant coagulase-negative staphylococci (MRCoNS), and vancomycin-resistant enterococci (VRE). To avoid increasing resistance, it is not advisable to commence antibiotics without clinical evidence of a UTI (exceptions include asymptomatic bacteriuria in pregnancy) and local microbiology guidelines should be followed.

Once urine or blood culture results are available, antimicrobial therapy should be adjusted according to bacterial sensitivities. Underlying abnormality should be corrected if feasible (i.e. extraction of infected calculus; removal of catheter; nephrostomy drainage of an infected, obstructed kidney). Post-menopausal women may benefit from topical oestrogen treatment.

Encourage a good fluid intake, cranberry juice, double voiding, avoid constipation. In women—voiding before and after intercourse; wiping perineum from ‘front to back’ after voiding; avoid using bubble bath or washing hair in the bath (as this affects the protective commensal organisms, the lactobacilli).

Recurrent UTI is defined as >2 infections in 6 months or 3 within 12 months. It may be due to re-infection (i.e. infection by different bacteria) or bacterial persistence (infection by the same organism originating from a focus within the urinary tract).

Bacterial persistence usually leads to frequent recurrence of infection (within days or weeks) and the infecting organism is usually the same organism as that causing the previous infection(s). There is often an underlying functional or anatomical problem and infection will often not resolve until this has been corrected. Causes include kidney stones, the chronically infected prostate (chronic bacterial prostatitis), bacteria within an obstructed or atrophic infected kidney, vesicovaginal or colovesical fistula, and bacteria within a urethral diverticulum.

This usually occurs after a prolonged interval (months) from the previous infection and is often caused by a different organism than the previous infecting bacterium.

Women: with re-infection, do not usually have an underlying functional or anatomical abnormality. Re-infections are associated with increased vaginal mucosal receptivity for uropathogens and ascending colonization from faecal flora. These women cannot be cured of their predisposition to recurrent UTIs, but they can be managed by a variety of techniques (see  p. 187).

Men: with re-infection, may have underlying BOO (due to BPE or a urethral stricture), which makes them more likely to develop a repeat infection, but between infections, their urine is sterile (i.e. they do not have bacterial persistence between symptomatic UTIs). A flexible cystoscopy, post-void bladder USS for residual urine volume and in some cases, urodynamics or urethrography may be helpful in establishing the potential causes.

Both men and women with bacterial persistence usually have an underlying functional or anatomical abnormality and they can potentially be cured of their recurrent UTIs if this abnormality can be identified and corrected.

Imaging tests, including KUB X-ray and renal USS, and flexible cystoscopy, can be performed to check for potential sources of bacterial persistence (i.e. to confirm this is a ‘simple’ case of re-infection rather than one of bacterial persistence). In the absence of finding an underlying functional or anatomic abnormality, these patients cannot be cured of their tendency to recurrent urinary infection, but they can be managed in several ways.

Oral antimicrobial therapy with full-dose oral tetracyclines, ampicillin, sulphonamides, amoxicillin, and cefalexin causes resistant strains in the faecal flora and subsequent resistant UTIs. However, trimethoprim, nitrofurantoin, and low-dose cefalexin have minimal adverse effects on the faecal and vaginal flora.

Sexual intercourse has been established as an important risk factor for acute cystitis in women and women using the diaphragm have a significantly greater risk of UTI those using other contraceptive methods.³ Post-intercourse therapy with antimicrobials, such as nitrofurantoin, cefalexin, or trimethoprim, taken as a single dose effectively reduces the incidence of re-infection.

Women keep a home supply of an antibiotic (e.g. trimethoprim, nitrofurantoin, or a fluoroquinolone) and start treatment when they develop symptoms suggestive of UTI.

These are directed at identifying the potential causes of bacterial persistence outlined on  p. 186.

This depends on the functional or anatomical abnormality that is identified as the cause of the bacterial persistence. If a stone is identified, this should be removed. If there is obstruction (e.g. BOO, PUJO, DSD in spinal injured patients), this should be corrected.

Definition: pyelonephritis is an inflammation of the kidney and renal pelvis.

Clinical diagnosis is based on the presence of fever, flank pain, bacteriuria, pyuria, often with an elevated white cell count. Nausea and vomiting are common. It may affect one or both kidneys. There are usually accompanying symptoms suggestive of a lower UTI (frequency, urgency, suprapubic pain, urethral burning or pain on voiding) responsible for the subsequent ascending infection to the kidney.

Differential diagnosis: includes cholecystitis, pancreatitis, diverticulitis, appendicitis.

Risk factors: females > males, VUR, urinary tract obstruction, calculi, SCI (neuropathic bladder), diabetes mellitus, congenital malformation, pregnancy, indwelling catheters, urinary tract instrumentation.

Pathogenesis and microbiology: initially, there is patchy infiltration of neutrophils and bacteria in the parenchyma. Later changes include the formation of inflammatory bands extending from the renal papilla to cortex and small cortical abscesses. Eighty percent of infections are secondary to E. coli (possessing P pili virulence factors). Other infecting organisms: Enterococci (E. faecalis), Klebsiella, Proteus, Staphylococci, and Pseudomonas. Any process interfering with ureteric peristalsis (i.e. obstruction) may assist in retrograde bacterial ascent from bladder to kidney.

An infected hydronephrosis where pus accumulates within the renal pelvis and calyces. It is associated with damage to the parenchyma, resulting in loss of renal function. The causes are essentially those of hydronephrosis where infection has supervened (e.g. ureteric obstruction by stone, PUJ obstruction).

Patients with pyonephrosis are usually very unwell with a high fever, flank pain, and tenderness.

Stone disease, previous UTI, or surgery.

IV fluids and antibiotics (as for pyelonephritis) with urgent percutaneous drainage (nephrostomy) or ureteric drainage (via ureteric catheter under endoscopic and X-ray guidance).

Perinephric abscess develops as a consequence of extension of infection outside the parenchyma of the kidney in acute pyelonephritis, from rupture of a cortical abscess, or if obstruction in an infected kidney (i.e. pyonephrosis) is not drained quickly enough. More rarely, it is due to haematogenous spread of infection from a distant site or infection from adjacent organs (i.e. bowel). The abscess develops within Gerota’s fascia.

Diabetes mellitus; immunocompromise; obstructing ureteric calculus may precipitate the development of a perinephric abscess.

Perinephric abscesses are caused by S. aureus (Gram-positive), E. coli, and Proteus (Gram-negative organisms).

Patients present with fever, unilateral flank tenderness, and ≥5 day history of milder symptoms. Failure of a seemingly straightforward case of acute pyelonephritis to respond to IV antibiotics within a few days also arouses suspicion that there is an accumulation of pus in or around the kidney or obstruction with infection.

A flank mass with overlying skin erythema and oedema may be observed. Extension of the thigh (stretching the psoas) may trigger pain and psoas spasm may cause a reactive scoliosis.

Commence broad-spectrum IV antibiotics (i.e. aminoglycoside and aminopenicillin with B-lactamase inhibitor) until culture sensitivities are available. Drainage of the collection should be performed, either radiographically or by formal open incision and drainage if the pus collection is large. IV antibiotics should be used initially and followed by a course of oral antimicrobials until clinical review and re-imaging confirms resolution of infection. Nephrectomy may be required for extensive renal involvement or a non-functioning infected kidney.

Maintaining a degree of suspicion in all cases of presumed acute pyelonephritis is the single most important thing in allowing an early diagnosis of complicated renal infection such as a pyonephrosis, perinephric abscess, or emphysematous pyelonephritis to be made. If the patient is very unwell, is diabetic, or has a history suggestive of stones, they may have something more than just a simple acute pyelonephritis. Specifically ask about a history of sudden onset of severe flank pain a few days earlier, suggesting the possibility that a stone passed into the ureter, with later infection supervening. Arranging a KUB X-ray and renal USS in all patients with suspected renal infection will demonstrate the presence of hydronephrosis, pus, or stones.

Clinical indicators suggesting a more complex form of renal infection are length of symptoms prior to treatment and time taken to respond to treatment. Most patients with uncomplicated acute pyelonephritis have been symptomatic for <5 days. Most with, for example, a perinephric abscess have been symptomatic for >5 days prior to hospitalization. Patients with acute pyelonephritis became afebrile within 4–5 days of treatment with an appropriate antibiotic whereas those with perinephric abscesses remained pyrexial.¹

A rare severe form of acute necrotizing pyelonephritis caused by gas-forming organisms. It is characterized by fever and abdominal pain, with radiographic evidence of gas within and around the kidney (on plain radiography or CT) (Fig. 6.1). It usually occurs in diabetics (93% in a contemporary series)¹ and, in many cases, is precipitated by urinary obstruction by, for example, ureteric stones. The high glucose levels associated with poorly controlled diabetes provides an ideal environment for fermentation by Enterobacteria, carbon dioxide being produced during this process. EPN is commonly caused by E. coli, less frequently by Klebsiella and Proteus.

Severe acute pyelonephritis (high fever and systemic upset) that fails to respond to IV antibiotics within 2–3 days.

KUB X-ray may show a crescent or kidney-shaped distribution of gas around the kidney. Renal USS often demonstrates strong focal echoes, indicating gas within the kidney. CT can help classify the disease. Type I shows parenchymal destruction, an absence of fluid collection, or streaky gas from the medulla to cortex—this has a poorer prognosis. Type II shows intrarenal gas and renal or perirenal fluid, or collecting system gas—this has a better prognosis.

Patients with EPN are usually very unwell (to the extent that many are not fit enough for emergency nephrectomy) and mortality is high. Resuscitate and transfer to ITU/HDU. In recent years, management has moved away from emergency nephrectomy to an approach with IV antibiotics, IV fluids, percutaneous drainage, and careful control of diabetes.¹ Where there is no symptomatic improvement, have a low threshold for rescanning (CT) and consider additional percutaneous drainage for ‘pockets’ of infection that have not been adequately drained.¹ In those where sepsis is poorly controlled, emergency nephrectomy may be required.

A severe renal infection, usually (although not always) occurring in association with underlying renal calculi and renal obstruction. Three forms exist: focal (XGP in the renal cortex with no pelvic communication), segmental, and diffuse. The severe infection results in the destruction of renal tissue, leading to a non-functioning kidney. E. coli and Proteus are common causative organisms. Lipid-laden, ‘foamy’ macrophages become deposited around abscesses within the parenchyma of the kidney. The infection may be confined to the kidney or extend to the perinephric fat. The kidney becomes grossly enlarged and macroscopically contains yellowish nodules (pus) and areas of haemorrhagic necrosis. It can be very difficult to distinguish the radiological findings from a renal cancer on imaging studies such as CT. Indeed, in most cases, the diagnosis is made after nephrectomy for what was presumed to be a renal cell carcinoma.

Acute flank pain, fever, haematuria, LUTS, and a tender flank mass. It affects all age groups, females more often than males.

Fistula (nephrocutaneous, nephrocolonic), paranephric abscess, psoas abscess.

Blood tests show anaemia and leukocytosis. Bacteria (E. coli, Proteus) may be found on culture urine. Renal USS shows an enlarged kidney containing echogenic material. CT may identify (obstructing) renal or urinary tract calculi, hydronephrosis, renal cortical thinning, and perinephric fat inflammation. Non-enhancing cavities are seen, containing pus and debris. On radioisotope scanning (DMSA, MAG3 renogram), there may be some or no function in the affected kidney.

On presentation, these patients are usually commenced on antibiotics as the constellation of symptoms and signs suggest infection. If systemically unwell, transfer to ITU/HDU for treatment. When imaging studies are done, such as CT, the appearances usually suggest the possibility of a renal cell carcinoma and, therefore, when signs of infection have resolved, the majority of patients will proceed to nephrectomy. Often, only following pathological examination of the removed kidney will it become apparent that the diagnosis was one of infection (XGP) rather than tumour.

In essence, this describes renal scarring which may or may not be related to previous UTI. It is a radiological, functional, or pathological diagnosis or description.

A child with VUR, particularly where there is reflux of infected urine, will develop reflux nephropathy (which, if bilateral, may cause renal impairment or renal failure). If the child’s kidneys are examined radiologically (or pathologically if they are removed by nephrectomy), the radiologist or pathologist will describe the appearances as those of ‘chronic pyelonephritis’.

An adult may also develop radiological and pathological features of chronic pyelonephritis due to the presence of reflux or BOO combined with high bladder pressures, again particularly where the urine is infected. This was a common occurrence in male patients with SCI and DSD before the advent of effective treatments for this condition.

Chronic pyelonephritis is essentially the end result of longstanding reflux (non-obstructive chronic pyelonephritis) or of obstruction (obstructive chronic pyelonephritis). These processes damage the kidneys, leading to scarring and the degree of damage and subsequent scarring is more marked if infection has supervened.

Patients may be asymptomatic or present with symptoms secondary to renal failure. Diagnosis is often from incidental findings during general investigation. There is usually no active infection.

Scars can be ‘seen’ radiologically on a renal USS, IVU, renal isotope scan, or CT. The scars are closely related to a deformed renal calyx. Distortion and dilatation of the calyces is due to scarring of the renal pyramids. These scars typically affect the upper and lower poles of the kidneys because these sites are more prone to intrarenal reflux. The cortex and medulla in the region of a scar is thin. The kidney may be so scarred that it becomes small and atrophic.

Aim to investigate and treat any infection, prevent further UTI, and monitor and optimize renal function.

Renal impairment progressing to end-stage renal failure in bilateral cases (usually only if chronic pyelonephritis is associated with an underlying structural or function urinary tract abnormality).

is the presence of pathogenic organisms in the bloodstream. This can lead to septicaemia or sepsis—the clinical syndrome caused by bacterial infection of the blood. This is confirmed by positive blood cultures for a specific organism and accompanied by a systemic response to the infection known as the systemic inflammatory response syndrome (SIRS). SIRS is defined by at least two of the following:

Septicaemia is often accompanied by endotoxaemia—the presence of circulating bacterial endotoxins.

Severe sepsis: sepsis associated with organ dysfunction (hypoperfusion or hypotension). Hypoperfusion and perfusion abnormalities may include lactic acidosis, oliguria, or acute altered mental state.

Septic shock: sepsis with hypotension¹ despite adequate fluid resuscitation with perfusion abnormalities that may include lactic acidosis, oliguria, or acute altered mental state. It results from Gram-positive bacterial toxins or Gram-negative endotoxins which trigger the release of cytokines (TNF, IL-1), vascular mediators, and platelets, resulting in vasodilatation (manifest as hypotension) and disseminated intravascular coagulation (DIC).

Refractory shock: is defined as septic shock (lasting >1h) which fails to respond to therapy (fluids or pharmacotherapy).

In the hospital setting, the most common causes are the presence or manipulation of indwelling urinary catheters, urinary tract surgery (particularly endoscopic—TURP, TURBT, ureteroscopy, PCNL), and urinary tract obstruction (particularly that due to stones obstructing the ureter). Septicaemia occurs in approximately 1.5% of men undergoing TURP. Diabetic patients, patients in ITU, and immunocompromised patients (on chemotherapy and steroids) are more prone to urosepsis.

Causative organisms in urinary sepsis:  E. coli, Enterococci, Staphylococci, Pseudomonas, Klebsiella, and Proteus.

The principles of management include early recognition, resuscitation, localization of the source of sepsis, early and appropriate antibiotic administration, and removal of the primary source of sepsis. From a urological perspective, the clinical scenario is usually a post-operative patient who has undergone TURP or surgery for stones. On return to the ward, they become pyrexial, start to shiver (chills) and shake, and are tachycardic and tachypnoea (leading initially to respiratory alkalosis). They may be confused and oliguric. They may initially be peripherally vasodilated (flushed appearance with warm peripheries). Consider the possibility of a non-urological source of sepsis (e.g. pneumonia). If there are no indications of infection elsewhere, assume the urinary tract is the source of sepsis.

This is ‘blind’ use of antibiotics based on an educated guess of the most likely pathogen that has caused the sepsis. Gram-negative aerobic rods are common causes of urosepsis (e.g. E. coli, Klebsiella, Citrobacter, Proteus, and Serratia). The enterococci (Gram-positive aerobic non-haemolytic Streptococci) may sometimes cause urosepsis. In urinary tract operations involving the bowel, anaerobic bacteria may be the cause of urosepsis and in wound infections, staphylococci (e.g. S. aureus and S. epidermidis) are the usual cause.

Refer to your local microbiology guidelines. Options include:

If there is clinical improvement, parenteral treatment (IV) should continue for 3–5 days after the infection has been controlled (or complicating factor has been eliminated), followed by a course of oral antibiotics. Make appropriate adjustments when sensitivity results are available from urine cultures (which may take about 48h).

Mortality rate: 13% with septicaemia alone; 28% with septicaemia and shock; 43% with septicaemia followed by septic shock.²

A necrotizing fasciitis of the external genitalia and perineum, primarily affecting males and causing necrosis and subsequent gangrene of infected tissues. Also known as spontaneous fulminant gangrene of the genitalia, it is a urological emergency.

Culture of infected tissue reveals a combination of aerobic (E. coli, enterococci, Klebsiella) and anaerobic organisms (Bacteroides, Clostridium, micro-aerophilic streptococci) which are believed to grow in a synergistic fashion.

Fournier’s gangrene is usually related to an initial genitourinary tract infection, skin trauma, or from direct extension from a perirectal focus. Spread of infection is through the local fascia (Buck’s fascia in the penis, Darto’s fascia in the scrotum, Colle’s fascia in the perineal region, and Scarpa’s fascia of the anterior abdominal wall). Infection produces tissue necrosis that can spread rapidly and pus produced by anaerobic pathogens (Bacteroides) produces the typical putrid smell.

A previously well patient may become systemically unwell following a seemingly trivial injury to the external genitalia. Early clinical features include localized skin erythema, tenderness and oedema, and sometimes with LUTS (dysuria, difficulty voiding, urethral discharge). This progresses to fever and sepsis with cellulitis and palpable crepitus in the affected tissues, indicating the presence of subcutaneous gas produced by gas-forming organisms. As the infection advances, blisters (bullae) appear in the skin and within a matter of hours, areas of necrosis may develop, which spread to involve adjacent tissues (e.g. lower abdominal wall).

The diagnosis is a clinical one and is based on the awareness of the condition and a high index of suspicion. In early stages of disease, abdominal X-ray, and scrotal USS, or CT may demonstrate the presence of air in tissues. CT can also indicate the extent of disease, however, most surgeons would not delay to image the patient, but progress directly to surgical treatment.

Mortality is in the order of 20–30%. Mortality rates are reported to be higher in patients with a degree of immunocompromise (diabetics, alcohol excess) and those with anorectal or colorectal disease/involvement. Mortality risk can be assessed by the Fournier’s gangrene severity index (FGSI)² based on nine clinical parameters: respiratory rate, heart rate, temperature, WBC count, haematocrit, sodium, potassium, creatinine, and sodium bicarbonate levels. Each parameter was valued between 0 and 4, with the higher value given to the greatest deviation from normal. FGSI >9 correlates with increased mortality (46–75%);^2,3 FGSI <9 has a reported 78–96% chance of survival.^2,3

Peri-urethral abscess can occur in patients with urethral stricture disease following urethral catheterization and in association with gonococcal urethritis. The bulbar urethra is a commonly affected site in men. These conditions predispose to bacteria (Gram-negative rods, enterococci, anaerobes, gonococcus) gaining access through Buck’s fascia to the peri-urethral tissues. If not rapidly diagnosed and treated, infection (necrotizing fasciitis) can spread to the perineum, buttocks, and abdominal wall. In immunocompromised patients (i.e. patients with HIV infection), M. tuberculosis is also a causative organism.

Extravasation of urine from the abscess cavity may result in cellulitis and a risk of fistula formation.

Emergency treatment is required. The abscess should be incised and drained, a suprapubic catheter placed to divert the urine away from the urethra, and broad-spectrum parenteral antibiotics commenced (gentamicin and cephalosporin) until antibiotic sensitivities are known. Any devitalized and necrotic tissue requires immediate surgical debridement.

An inflammatory condition of the epididymis, often also involving the testis, and usually caused by bacterial infection. It has an acute onset and a clinical course lasting <6 weeks, presenting with epididymal pain, swelling, and tenderness. It can occur in all age groups.

Infection ascends from the urethra or bladder. In sexually active men aged <35y, the infective organism is usually N. gonorrhoeae, C. trachomatis, or coliform bacteria (causing a urethritis which then ascends to infect the epididymis). In older men and children, the infective organisms are usually common uropathogens (i.e. E. coli). M. tuberculosis (TB) is a rarer cause of epididymitis where the epididymis feels like a ‘beaded’ cord (see  p. 220).

A rare, non-infective cause of epididymitis is the antiarrhythmic drug, amiodarone, which accumulates in high concentrations within the epididymis, causing inflammation.¹ It can be unilateral or bilateral and resolves on discontinuation of the drug. Some cases of epididymitis in children are also non-infective (idiopathic or as a result of trauma).

Fever; testicular swelling; scrotal pain that may radiate to the groin (spermatic cord) and lower abdomen; erythema of scrotal skin; thickening of spermatic cord; reactive hydrocele; evidence of underlying infection (urethral discharge, symptoms of urethritis, cystitis, or prostatitis).

If any doubt in the diagnosis exists, exploration is the safest option. Although radionuclide scanning can differentiate between a torsion and an epididymitis, this is not widely available. Colour Doppler USS, which provides a visual image of blood flow, can differentiate between a torsion and epididymitis, but its sensitivity for diagnosing torsion is only 80% (i.e. it ‘misses’ the diagnosis in 20% of cases). Its sensitivity for diagnosing epididymitis is about 70%.

Bed rest, analgesia, scrotal elevation, and antibiotics.

Until culture sensitivities are available and where C. trachomatis is a possible infecting organism, prescribe a 14-day course of doxycycline 100mg twice daily (an alternative is a single dose of azithromycin 1g). If gonorrhoea is suspected or confirmed, prescribe ciprofloxacin (500mg bd for 14 days). An alternative is a single dose of oral cefixime (400mg). Patients should be referred for genitourinary medicine (GUM) input and tracing of sexual contacts.

For non-sexually transmitted infection of the epididymis, prescribe antibiotics empirically (until culture results are available) according to your local microbiology department advice. Our empirical antibiotic regimen is ciprofloxacin for 2 weeks. When the patient is systemically unwell, we admit them for IV cephalosporin and IV gentamicin (initially 3–5mg/kg, then adjusted according to serum gentamicin concentration). When the patient becomes apyrexial, we change to oral ciprofloxacin for 2 weeks. Any underlying cause of infection should be identified and treated (i.e. BOO) to prevent further episodes.

These include abscess formation (requiring incision and drainage), infarction of the testis, chronic pain and infection, and infertility.

Diagnosed in patients with long-term pain in the epididymis (9 testicle). It can result from recurrent episodes of acute epididymitis. Clinically, the epididymis is thickened and may be tender. Treatment is with the appropriate antibiotics (guided by cultures) and analgesia. Epididymectomy is reserved for severe refractory cases.

Orchitis is inflammation of the testis, although it often occurs with epididymitis (epididymo-orchitis). Causes include the mumps virus, M. tuberculosis, syphilis, autoimmune processes (granulomatous orchitis). The testis is swollen and tense, with oedema of connective tissues and inflammatory cell infiltration. Treat the underlying cause.

Mumps orchitis occurs in 30% of infected post-pubertal males. It manifests 3–4 days after the onset of parotitis and can result in tubular atrophy. Ten to thirty percent of cases are bilateral and are associated with testicular atrophy and infertility.

Prostatitis is infection and/or inflammation of the prostate, which is described as acute or chronic and bacterial or abacterial. The classification system is from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institute of Health (NIH) in the United States. Chronic abacterial prostatitis is further divided into inflammatory and non-inflammatory types, guided by the results of segmented urine cultures.

Technique described by Meares and Stamey (1968) to help classify the type of prostatitis. It localizes bacteria to a specific part of the urinary tract by sampling different parts of the urinary stream with prostatic massage which produces EPS. Where cultures are negative, increased numbers of leukocytes per high powered field (>10) on microscopy favours a diagnosis of inflammatory CPPS.

Thirty minutes before the test, the patient should drink 400mL of fluid. Retract foreskin and cleanse the glans before specimen collection.

Prostatitis is estimated to affect 50% of men at some point in their lives. The overall prevalence is reported at 5–14%. Age groups at increased risk are 20–50y and >70y.

The most common infective pathogens are Gram-negative Enterobacteriaceae (E. coli in 80% of cases, Klebsiella, Proteus, Pseudomonas). Both type 1 and P pili are important bacterial virulence factors that facilitate infection. Five to ten percent of infections are caused by Gram-positive bacteria (S. aureus and S. saprophyticus, E. faecalis). Acute bacterial prostatitis is often secondary to infected urine refluxing into prostatic ducts that drain into the posterior urethra. The resulting oedema and inflammation may then obstruct the prostatic ducts, trapping uropathogens and causing progression to chronic bacterial prostatitis in 75%.

The underlying aetiology is not fully understood, but is likely to be multifactorial. The Multidisciplinary Approach to Pelvic Pain (MAPP) research project has been set up to evaluate the importance and impact of various ‘clinical phenotypes’ for CPPS. Essentially, patients may have a predominance of certain symptoms or conditions that feature in their disease, suggestive of the main underlying aetiology (i.e. neurological, endocrine, immunological, infectious, neuromuscular, and psychosocial components). The MAPP study aims to identify potential biomarkers relating to these ‘clinical phenotypes’ which will ultimately help with the diagnosis and direct patient specific management.

Acute infection of the prostate associated with lower urinary tract infection and generalized sepsis. The underlying focus or cause of initial infection should be identified and also treated (i.e. BOO, urethral stricture, voiding dysfunction, urinary tract stones).

Factors that predispose to genitourinary tract and then prostatic colonization with bacteria are:

Further investigation is guided by individual patient presentation and clinical suspicion. Although segmented urine cultures are recommended in some guidelines, prostatic massage should be avoided in the acute, painful phase of prostatitis.

Failure to respond to treatment (i.e. persistent symptoms and fever while on appropriate antibiotic therapy) suggests the development of a prostatic abscess. The majority are due to E. coli infection. Risk factors include diabetes mellitus, immunocompromise, renal failure, transurethral instrumentation, and urethral catheterization. Rectal examination demonstrates a tender, boggy-feeling prostate or an area of fluctuance. A transrectal USS or CT scan (if the former proves too painful) is the best way of diagnosing a prostatic abscess. Transurethral resection or deroofing of the abscess is the optimal treatment. Alternatively, percutaneous drainage may be attempted.

Defined as bacterial prostatitis where symptoms persist for ≥3 months.

Caused by recurrent UTI. Chronic episodes of pain, voiding dysfunction, and ejaculatory problems may be a feature.

Enquire about factors that may be contributing to infection: urinary symptoms, history of renal tract stones, symptoms suggesting a colovesical fistula in at-risk patients (pneumaturia, history of diverticular disease, pelvic surgery, or radiotherapy). DRE may reveal a tender, enlarged, and boggy prostate.

Refers to abacterial prostatitis (i.e. inflammatory (III_A) and non-inflammatory (III_B) types of prostatitis). Also referred to as ‘prostate pain syndrome’. The aetiology and pathophysiology is unknown.

Some groups of patients will benefit more from specific therapies than others. Patients require a multimodal approach to treatment, guided by their main clinical features.¹ Options include:

If no pathology is identified and there is no response to initial treatments, referral to the pain team is advised.

A chronic and debilitating disorder characterized by urinary frequency, urgency, nocturia, and bladder and pelvic pain. It remains a diagnosis of exclusion after all other causes for the symptoms have been ruled out (Table 6.5). The ‘classic’ form is associated with bladder ulceration (Hunner’s ulcers) and destructive inflammation, with some developing a small-capacity fibrotic bladder or upper urinary tract outflow obstruction. ‘Non-ulcer’ forms do not show the same progression.

Terminology has changed a number of times. It was formally known as interstitial cystitis (IC). The ICS, the European Society for the Study of Bladder Pain Syndrome/Interstitial Cystitis (ESSIC), and the EAU use the term ‘BPS’.^1,2 The AUA use the term ‘IC/BPS’.^2,3

ESSIC: ‘chronic (>6 months) pelvic pain, pressure, or discomfort perceived to be related to the urinary bladder, accompanied by at least one other urinary symptom such as persistent urge to void or frequency’.^1,2

AUA: ‘an unpleasant sensation (pain, pressure, discomfort) perceived to be related to the urinary bladder, associated with LUTS >6 weeks, in the absence of infection or other identifiable causes’.^2,3

Predominantly affects females (female : male ratio is >5:1). Reported prevalence varies widely, but is estimated to be 300 per 100 000 women and 30–60 per 100 000 men.²

Irritable bowel syndrome, allergies, fibromyalgia, chronic fatigue syndrome, focal vulvitis, Sjögren’s syndrome, inflammatory bowel disease.

BPS is now considered a generalized somatic disorder with multifactorial contributing factors, including:

Urinary frequency, urgency and nocturia with associated suprapubic pain, pressure or discomfort related to bladder filling (and typically relieved by bladder emptying). Patients often describe pelvic pain (urethra, vagina, vulva, rectum) and pain in the lower abdomen and back.

The first priority is to exclude other causes for symptoms (Table 6.5).

Further investigations (if clinically indicated):

There should be a multidisciplinary team approach throughout from physicians, dieticians, physiotherapists, pain specialists, psychologists, and patient support groups.

Of note, it is recommended that patients are not given: long-term antibiotics in the absence of proven infection or effectiveness; intravesical BCG; intravesical resiniferatoxin; high-pressure, long duration hydrodistension; or long-term oral glucocorticoids.

Ketamine is a sedative and an analgesic used clinically for the induction and maintenance of anaesthesia. Increasingly, it is being misused as a recreational drug (its class C status is under review). It is a non-competitive N-methyl-D-aspartate receptor antagonist, excreted with its metabolites into the urine. It has hallucinogenic effects, producing an out-of-body experience known as the ‘K-hole’.

Inflammation of bladder epithelium, denuding of urothelium, and neovascularization and petechial haemorrhage of the bladder are reported.¹ Histologically, it may mimic the characteristics of carcinoma in situ.² The exact mechanism of damage is not known. Theories include a direct toxic effect of ketamine and its metabolites on the genitourinary tract, microvascular reactions and damage, and autoimmune processes.

An association of these symptoms with hepatic dysfunction is reported.³

A full history of urinary symptoms and recreational drug use is essential, including doses and duration of use, as they impact on prognosis. Patients often use other substances at the same time.

Tuberculosis (TB) of the genitourinary tract is caused by Mycobacterium (M.) tuberculosis. TB was formerly predominantly seen in Asian populations, but is now seen with increasing incidence in those from other ethnic groups and immunocompromised patients (i.e. with HIV infection). It has a higher incidence in males than females.

Primary TB: the primary granulomatous lesion forms in the mid to upper zone of the lung. It consists of a central area of caseation surrounded by epitheloid and Langhans’ giant cells, accompanied by caseous lesions in the regional lymph nodes. There is early spread of bacilli via the bloodstream to the genitourinary tract, but immunity rapidly develops and the infection remains quiescent. Acute diffuse systemic dissemination of tubercle bacilli can result in symptomatic miliary TB.

Post-primary TB: reactivation of infection is triggered by immune compromise (including HIV). It is at this point that patients develop clinical manifestations.

Early symptoms include fever, lethargy, weight loss, night sweats, and UTI not responding to treatment. Later manifestations include LUTS, haematuria, and flank pain.

A multidisciplinary team approach is required, involving colleagues from respiratory, infectious diseases, and microbiology departments. Treatment is with 2 months of isoniazid, rifampicin, and pyrazinamide and ethambutol, followed by a continuation phase of 4 months of isoniazid and rifampicin. Longer treatments or modification of drugs is needed for complications and resistant organisms.

A non-functioning, calcified kidney may need nephrectomy. Regular follow-up imaging with IVU is recommended to monitor for ureteric strictures which may need stenting, nephrostomies, or ureteric reimplantation. Severe bladder disease may require surgical augmentation, urinary diversion or cystectomy, and neobladder reconstruction. For epididymal involvement, epididymectomy ± orchidectomy is considered if pharmacotherapy fails or extensive disease is present.

This is caused by the parasitic trematode (or flatworm) called Schistosoma (S.) haematobium. It occurs in Africa (Egypt) and the Middle East. Other causes of schistosomiasis include S. mansoni, S. japonicum, S. mekongi, and S. intercalatum. They are mainly responsible for intestinal forms of disease.

Infection is acquired by exposure to contaminated water. The parasite (cercariae form) penetrates the skin of the human host, shed their tails, and become schistosomula. They migrate first to the lung via venous circulation, then to the liver to mature. The adult worms couple (sexual reproductive phase), migrate to veins of the vesical plexus, and lay fertilized eggs. Most eggs (which typically have a terminal spine) leave the body by penetrating the bladder and entering the urine. Some eggs are trapped in the tissues and those not destroyed by host responses can become calcified. The released eggs hatch in fresh water, releasing miracidia which find and enter the intermediate host, a fresh water Bulinus species snail. Through an asexual reproductive phase, sporocytes are created in the snail. These produce and later release larvae called cercariae, the free-swimming, infective form of the parasite, and the cycle is continued, with penetration into the human host. The disease has two main stages: active (when adult worms are laying eggs) and inactive (when the adults have died and there is a reaction to the remaining eggs).

Lesions occur due to calcification of dead eggs trapped in tissues, triggering a fibrotic reaction. A T-cell-mediated immune response is stimulated also by the presence of the eggs, resulting in eosinophilic granuloma in the bladder, uterus, and genitalia.

Praziquantel 40mg/kg as a single or divided oral doses. Corticosteroids are an adjuvant therapy used to treat Katayama fever (within 2 months of freshwater contact). Patients should be followed up at 2 and 6 months with urinalysis and clinical assessment.

Lymphatic filariasis caused by Wuchereria bancrofti infection is common in the tropics and is transmitted by mosquitoes. Genitourinary manifestations, which may be delayed up to 5y, include funiculoepididymitis, orchitis, hydrocoele, scrotal and penile elephantitis, and lymph scrotum (oedema). Diagnosis is on thick film, serology, or biopsy. Medical treatment is with diethylcarbamazine. Surgical excision of fibrotic and oedematous tissue may be needed for genital elephantitis.

Causes a spectrum of illness related to immune system deficiency. HIV-1 is pandemic and accounts for significant mortality in developing countries. HIV-2 has less pathogenicity and is predominant in West Africa. Transmission is via unprotected sexual intercourse, contaminated needles, mother-to-fetus transmission, infected blood, and blood products (blood transfusion risks are now minimal).

HIV is a retrovirus. It possesses the enzyme, reverse transcriptase, that enables viral RNA to be transcribed into DNA, which is then incorporated into the host cell genome. HIV binds to CD4 receptors on helper T-lymphocytes (CD4 cells), monocytes, and neural cells. After an extended latent period (8–10y), CD4 counts decline. Acquired immunodeficiency syndrome (AIDS) is defined as HIV positivity and CD4 lymphocyte counts <200 x 10⁶/L. The associated immunosuppression increases the risk of opportunistic infections and tumours.

ELISA testing of serum detects antibodies against HIV antigens. The second confirmatory test is Western blot. Informed consent is required for the test.

Of note, circumcision has been shown to reduce the risk of acquiring HIV infection in heterosexual men.

The risk of HIV transmission after percutaneous exposure to HIV-infected blood is 3 per 1000 injuries.¹ Risks are increased if the patient has terminal HIV-related illness, a deep injury, visible blood on the device causing the injury, and injury with a needle previously placed in the source patient’s vein or artery.¹ After mucocutaneous exposure, the risk is <1 in 1000.¹

Immediately wash the area well and encourage free bleeding of puncture wounds. Irrigate exposed mucous membranes with water. Report to occupational health (A & E or equivalent out-of-hours service) for a risk assessment and baseline blood sample for storage. The source patient will be approached and counselled on undergoing HIV testing. Health care worker follow-up testing is recommended at 12 and 24 weeks post-exposure (or 24 weeks after antiretroviral prophylaxis if prescribed). Occupational HIV exposure is reported to the HPA Centre for Infections.

Post-exposure prophylaxis (PEP) is recommended if there has been significant occupational exposure to blood or another high-risk body fluid from a patient or other source, either known to be HIV-infected or considered high risk of HIV infection (but where the result of a HIV test has not or cannot be obtained). PEP should be initiated ideally within 1h and continued for at least 28 days. The PEP starter pack contains one Truvada tablet (tenofovir and emtricitabine) taken once a day plus two Kaletra tablets (lopinavir and ritonavir) taken twice a day.¹

A condition where the contracted foreskin (prepuce) has a tight, narrowed orifice and cannot be retracted back over glans of the penis. A physiological phimosis is present at birth due to adhesions between the epithelium of inner foreskin and glans. Penile growth, erections, and accumulation of epithelial debris (smegma) under the foreskin causes gradual separation. Ninety percent of foreskins are retractile at age 3.¹ Few persist into adulthood (<1% phimosis at age 17).² Recurrent balanitis and inflammatory conditions such as BXO in uncircumcised males can cause new pathological phimosis (see  p. 230).

Physiological phimosis is usually asymptomatic. Patients may describe ballooning of the foreskin on voiding and an inability to fully retract the foreskin which, in sexually active men, may also cause skin trauma during sexual intercourse. Inflammation or infection (balanitis and balanoposthisis) may cause bleeding, pain, discharge, or dysuria (see  p. 230). Phimosis associated with BXO presents with white, itching plaques affecting the foreskin and glans and may have associated voiding problems.

Adults: treat any associated infection. If symptomatic or a pathological phimosis, surgical treatment is circumcision (see  p. 750). Preputioplasty (longitudinal incision on foreskin which is closed transversely) is an alternative for milder cases. It is effective in around 50%; a circumcision is then required for those who do not respond. It is not suitable for BXO.

Children: older children with phimosis suffering infection (balanitis) can be treated with antibiotics and a course of topical 0.1% betamethasone (betnovate) cream which acts to soften the phimosis and allow foreskin retraction. The recommendations are to avoid circumcision where possible.¹

Indications for circumcision in children include phimosis associated with recurrent balanitis, BXO, (recurrent) UTI associated with an underlying abnormality (i.e. VUR,³ posterior urethral valves, neuropathic bladder dysfunction), recurrent UTI,³ failed medical therapy for UTI, stone disease, and for religious reasons.

Contraindications to (neonatal) circumcision include the presence of hypospadias (9 chordee or hooded foreskin), small penis, or large hernia or hydrocele (where repair after circumcision may cause a buried penis or secondary phimosis).

Balanitis is inflammation of the glans penis. Balanoposthitis is inflammation of the prepuce (foreskin) and glans. Increased risk with phimosis and uncircumcised males. Causes are shown in Table 6.6. Clinical features include pain, erythema, discharge, difficulty retracting the prepuce, and voiding dysfunction. Treat any proven infection, instruct on good hygiene, and avoid irritants. A short course of topical steroid cream (i.e. 0.1% bethamethasone) can be applied to improve retractability of the prepuce. Surgical options for recurrent infections include circumcision.

Also referred to as ‘plasma cell balanitis’. It tends to occur in older, uncircumcised men. Patients present with well-circumscribed, shiny, moist, erythematous plaque on the glans (sometimes described as having a ‘cayenne pepper’ appearance), with a corresponding lesion on the prepuce. Pathological features are chronic inflammatory cell (plasma cell) infiltrate in the dermis. It is usually asymptomatic, but may present with irritation, pain, or discharge. Differential diagnosis is erythroplasia of Queyrat, lichen planus, fixed drug eruption, or psoriasis and a skin biopsy is often indicated to confirm the diagnosis. A swab should be taken for microbiological analysis as secondary infection is common and requires antibiotic treatment. Conservative therapies include advice on hygiene, topical corticosteroids (9 antibiotics or antifungals as clinically indicated), but the disorder tends to persist or recur. Definitive treatment is with circumcision. Carbon dioxide laser therapy also has reported success.

It affects all age groups and can occur in isolation on the penis or as part of a generalized eruption. It presents as an itchy, papular rash. It consists of mauve papules which have a flat top covered in white streaks (Wickham’s striae). It affects flexor surfaces (wrists, elbows), genitalia (appearing as a white annular lesion or erythematous plaques on the glans penis), buccal mucosa, lumbar region, and ankles. The diagnosis is made clinically; biopsy can be used if the diagnosis is unclear or the lesions fail to respond to appropriate treatment. It is often self-limiting, but topical steroids can be prescribed for symptomatic lesions.

Chronic papulosquamous inflammatory skin disease, presenting with itchy pink plaques covered in silver white scales on hair-bearing areas and extensor surfaces (knees and elbows). It also causes pitting of the nails. Lesions may be guttate (raindrop-shaped), circinate (rings), or geographic. Genital psoriasis may present as itching and soreness of the groins and glans and a red penile rash. It may also involve the prepuce. It is treated with topical emollients, soap substitutes, and short courses of topical low-dose steroid creams.

The typical triad of symptoms is urethritis, conjunctivitis, and seronegative arthritis. It tends to present in younger men. It can be caused by STI (Chlamydia trachomatis) and there is an association with HIV. Penile, oral, and skin lesions may occur, which can be confused for psoriasis. Genital manifestations include circinate balanitis (ring-shaped eroded lesions on the glans penis) in uncircumcised men, which can appear as a crust lesion in circumcised patients. Patients should be under STI screening. The condition is self-limiting and usually resolves spontaneously.

An uncommon disorder of unknown cause characterized by painful genital (scrotum, prepuce, glans) and oral (aphthous) ulceration, polyarthritis, uveitis, and neurological syndromes. Treatment of genital lesions is with topical steroids (corticosteroids).