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scopic drainage with transgastric stents. The patient had slow resolution of liver dysfunction and was commenced on glucocorticoid therapy to manage residual IgG4 disease with good clinical progress and no further complications. Strictures of the hepatic hilum can pose a diagnostic difficulty. This case highlights IgG4-related sclerosing cholangitis (IgG4-SC), an extra-pancreatic manifestation of IgG4-related sclerosing disease (IRSD), as a differential for benign hepatic strictures. Hilar or intrahepatic ductal involvement, together with an associated softtissue mass in IgG4-SC presentation, can mimic hilar CCa presentation.1 Radical surgery for suspected CCa is considered the only option to prolong survival despite the associated post-operative morbidity.2 The vast majority of IgG4-SC cases are managed surgically for suspected biliary malignancy when there is no evidence of pancreatitis.3 Diagnosis of IgG4-SC has been aided by the development of HISORt (histology, imaging, serology, other organ involvement and response to therapy) criteria and Japanese IgG4-SC working group statement allowing for distinction from CCa.2,4 IRSD is characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis.5 Clinical symptomatology on presentation includes obstructive jaundice, weight loss, mild to moderate abdominal pain, steatorrhoea and new onset diabetes.4,6 Radiological features include diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with bile duct wall thickening.7 Although the utility of elevated serum IgG4 concentrations (>140 g/L) can be highly suggestive of IgG4-SC, it is not regarded pathognomonic as approximately 30% of patients have normal serum IgG4 concentrations.2,5,8 Other sensitive but not specific serological markers include hypergammaglobulinaemia, hyper IgG, antinuclear antibody, rheumatoid factor and peripheral eosinophilia.3 While co-presentation with autoimmune pancreatitis commonly occurs, IgG4-SC can occur as isolated biliary disease and can manifest as either a diffuse sclerosing cholangitis or a hilar pseudotumourous mass.1,8 Enlargement of the salivary or lacrimal gland, retroperitoneal fibrosis or renal lesion may also be noted on presentation.5 Given the unreliability of imaging modalities and brush cytology in distinguishing hepatic strictures, preoperative histological analysis is desired, through either Vater’s ampulla biopsy, liver needle biopsy or bile duct biopsy, with more than 10 IgG4-positive plasma cells used as the diagnostic threshold.3,8 Glucocorticoids are typically the first line of therapy, with most patients demonstrating a response within 4 weeks and treatment

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tapered over 3–6 months.4,5,7 This response is dependent on the degree of fibrosis within the affected organs, and monitored with a decline in serum biochemical markers and radiological improvement. Alternate treatment regimen includes the use of steroid sparing agents following steroid-induced remission and rituximab in patients with recurrent or refractory disease.6 In conclusion, this case highlights an extra-pancreatic manifestation of IgG4-related sclerosing disease and illustrates the diagnostic challenges faced in differentiating between benign and malignant biliary strictures.

References 1. Vlachou PA, Khalili K, Jang HJ, Fischer S, Hirschfield GM, Kim TK. IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. Radiographics 2011; 31: 1379–402. 2. Lytras D, Kalaitzakis E, Webster GJ et al. Cholangiocarcinoma or IgG4associated cholangitis: how feasible it is to avoid unnecessary surgical interventions? Ann. Surg. 2012; 256: 1059–67. 3. Zen Y, Nakanuma Y. IgG4 cholangiopathy. Int. J. Hepatol. 2012; 2012: Article ID 472376. doi: 10.1155/2012/472376. 4. Montano-Loza AJ, Lalor E, Mason AL. Recognizing immunoglobulin G4 related overlap syndromes in patients with pancreatic and hepatobiliary diseases. Can. J. Gastroenterol. 2008; 22: 840–6. 5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N. Engl. J. Med. 2012; 366: 539–51. 6. Alderlieste YA, van den Elzen BD, Rauws EA, Beuers U. Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion 2009; 79: 220–8. 7. Ohara H, Okazaki K, Tsubouchi H et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis. J. Hepatobiliary Pancreat. Sci. 2012; 19: 536–42. 8. Senthil Kumar MP, Marudanayagam R. Klatskin-like lesions. HPB Surg. 2012; 2012: Article ID 107519. doi: 10.1155/2012/107519.

Yogeesan Sivakumaran,* MBBS (Hons) Philip A. Le Page,* MBBS, FRACS Guillermo Becerril-Martinez,* MD William D. Beasley,* MBBS, MPhil, FRCS Lyndal A. Anderson,† MBBS, MPhil, MHM, FRCPA, FIAC David M. Joseph,* MBBS, FRACS Gregory L. Falk,* MBBS, FRACS, FACS *Department of Surgery, Concord Repatriation General Hospital, and †Department of Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia doi: 10.1111/ans.12251

Don’t miss it: a rare case of multifocal necrotizing fasciitis A 40-year-old obese woman presented to a peripheral hospital alert but in septic shock. She described a non-specific 3-day prodromal illness, which included myalgias, arthralgias and an increasingly painful right upper limb. She was previously healthy and denied any recent history of trauma, stings, bites or infectious symptoms. Appropriate cultures were taken, aggressive resuscitation com© 2013 Royal Australasian College of Surgeons

menced along with antibiotics (ceftriaxone and flucloxacillin), and she was transferred to the nearest tertiary intensive care unit (ICU). Within hours of transfer, her condition deteriorated, requiring intubation, increasing levels of haemodynamic support and haemofiltration for anuric renal failure and a mild acidosis (pH 7.31). She also had a marked thrombocytopenia (platelet count:

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Fig. 1. Contrast the diffusely mottled skin due to vasopressors with the dense purplish skin discoloration of the right arm resulting from a necrotic subcutaneous layer (bottom left).

58). Worsening purpura and bullae were noted to be affecting her right upper and lower limbs and the physicians discussed the possibility of a vasculitis, thrombotic thrombocytopenia purpura, haemolytic uraemic syndrome and a necrotizing soft tissue infection. Antibiotic therapy was broadened, and steroids and i.v. immunoglobulin commenced. Shortly thereafter, a positive blood culture for Group A Streptococcus (GAS) was returned from the initial hospital and a surgical opinion was sought. On examination, the patient was an overweight female with weak peripheral pulses and diffusely mottled, cold skin (Fig. 1). Purpura and large bullae were prominent features on the right lower leg and thigh and, to a lesser extent, the right forearm. The underlying soft tissue had a greyishblue discoloration hemicircumferentially (Figs 2,3). A diagnosis of multi-limb necrotizing fasciitis (NF) with toxic shock syndrome was made at the bedside and at operation. This was subsequently confirmed histologically. Extensive excision of necrotic fascia and muscle in all three compartments of her right leg and thigh followed, including a limited debridement of the forearm. At the end of the operation, the patient’s physiological state had improved markedly. The following morning, although still relatively physiologically stable, there was evidence of the infection spreading beyond the resection margins to the right groin and right arm, and new areas of patchy necrosis in her left upper and lower limbs. The need for extensive three limb amputations and hyperbaric therapy was discussed with the orthopaedic unit and explained to the family, with the general consensus being that no further intervention be undertaken. Twenty-four hours later, the patient died. NF is an uncommon but devastating disease process associated with an overall mortality rate approaching 20%.1 Those who survive are left with significant deformity and functional morbidity with little known about their long-term quality of life and psychological well-being.2 Diabetes, obesity, immunosuppression, GAS infection3 and surgical delay4 are associated with poorer outcomes; as is head and neck, trunk and multiple non-contiguous site involvement.5 Broad-spectrum antibiotics, aggressive surgical debridement and

Fig. 2. The red-purple skin discoloration with occasional haemorrhagic bullae indicating areas of necrotic underlying soft tissue is consistent with necrotizing fasciitis (NF) or myositis (e.g. clostridial myonecrosis). Duration of symptoms distinguishes the two with the latter presenting within hours of onset and NF typically over days as occurred in this case.

Fig. 3. (as for Fig. 2).

hyperbaric oxygen therapy is the only treatment and success depends on prompt clinical recognition. NF, however, is a notoriously challenging diagnosis. With its predilection for the deep fascia and subcutaneous tissue, the overlying skin and muscle beneath are initially spared. Established infec© 2013 Royal Australasian College of Surgeons

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tion then spreads freely along the suprafascial plane – an anatomically favourable dissection plane harnessed surgically in the elevation of skin flaps – providing a ‘path of least resistance’ and the rationale for why the true involved area may extend insidiously well beyond clinically identifiable boundaries. Further complicating the clinical picture, NF rarely may present at multiple non-contiguous sites. El-Khani et al.6 identified 31 citations for multifocal disease in the sole systematic review on the topic with one-third of the patients undergoing surgical debridement on average 7.8 days post admission, suggesting a delay in recognition. Small numbers and data heterogeneity limit what can be extrapolated from the series, but little appears to distinguish those from monofocal disease. There were, however, a number of notable observations. First and perhaps unsurprisingly, mortality (43%) was higher, likely attributable to the difficulty in achieving adequate surgical debridement. Secondly, in contrast to the trends seen in monofocal disease – where polymicrobial (type 1) infection predominates over monomicrobial (type 2) infection (80% versus 20%) – in multifocal NF, the incidence of monomicrobial, and in particular GAS infection, was much higher (52%). This is theoretically plausible. GAS virulence is well recognized being associated with a bacteraemia and toxic shock syndrome, and this systemic involvement would be consistent with septic embolization and disseminated infection.7 Lastly, there is often three-to-four limb involvement (15/33 cases in the series). In our case, non-contiguous right upper and lower limb lesions developed within 24 h of admission, and likely new septic lesions in the remaining limbs not 12 h subsequent to debridement. Subacute progression of the disease has been documented as late as 9 days post-admission. In our experience, the unusual presentation of the disease may have contributed to a delay in surgical referral and the time to definitive

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intervention. Prompt surgical referral for exploration/debridement of any patient with a similar clinical picture is recommended.

References 1. Anaya DA, McMahon K, Nathens AB, Sullivan SR, Foy H, Bulger E. Predictors of mortality and limb loss in necrotizing soft tissue infections. Arch. Surg. 2005; 140: 151–7. 2. Ruth-Sahd L, Gonzales M. Multiple dimensions of caring for a patient with acute necrotizing fasciitis. Dimens. Crit. Care Nurs. 2006; 25: 15–21. Review. 3. Darenberg J, Luca-Harari B, Jasir A et al. Molecular and clinical characteristics of invasive group A streptococcal infection in Sweden. Clin. Infect. Dis. 2007; 45: 450–8. 4. Wong CH, Chang HC, Pasupathy S, Khin LW, Tan JL, Low CO. Necrotizing fasciitis: clinical presentation, microbiology, and determinants of mortality. J. Bone Joint Surg. Am. 2003; 85-A: 1454–60. 5. Yong JM. Necrotising fasciitis. Lancet 1994; 343: 1427. 6. El-Khani U, Nehme J, Darwish A et al. Multifocal necrotizing fasciitis: an overlooked entity? J. Plast. Reconstr. Aesthet. Surg. 2012; 65: 501–12. 7. Stevens DL. Streptococcal toxic-shock syndrome: spectrum of disease, pathogenesis, and new concepts in treatment. Emerg. Infect. Dis. 1995; 1: 69–78.

Edwin John Morrison,* MBBS Benjamin P. C. Wei,† MBBS, PhD, FRACS Andrew K. Hadj,* MBBS Roger E. Adlard,* MBBS, MSc, FRSC (Plast) Wai-Ting Choi,* MBBS, FRACS *Western General Hospital and †St Vincent’s Hospital, Melbourne, Victoria, Australia doi: 10.1111/ans.12278

Radiation-induced necrosis and calcinosis of the breast: a case report Adjuvant radiotherapy decreases loco-regional recurrence rates following breast conserving surgery (BCS) for early breast cancer. It is used post-operatively in most cases of BCS for invasive disease and frequently for ductal carcinoma in situ (DCIS).1,2 Side effects of radiotherapy include chest wall discomfort, chronic pain, fatigue, breast lymphoedema, compromised cosmesis and possibly increased cardiovascular or lung disease.3 We report a rare side effect, a case study of radiotherapy-induced necrosis of the breast with associated calcinosis after BCS, which culminated in the patient requiring a total mastectomy. This patient initially presented in 1999, aged 78, with a screen detected right-sided breast cancer. There was a family history of breast cancer and a personal history of diabetes and gastrointestinal reflux disease. She underwent BCS and a level 2 axillary clearance in August 1999. Histopathology showed a 10-mm grade 2 infiltrating carcinoma of no special type and no lymph node involvement. Post-operative recovery was uneventful. She received adjuvant radiotherapy of 50 Gy in 25 fractions, with an additional 10 Gy in 5 © 2013 Royal Australasian College of Surgeons

fractions to the tumour bed. During radiotherapy treatment, she developed a severe skin reaction with an area of desquamation at the lateral edge. The breast was oedematous and indurated posttreatment. Two years post-operatively, she developed a large radionecrotic ulcer on the breast, which was successfully managed with dressings. She subsequently developed numerous smaller ulcers, which also healed with conservative measures. However, the breast became contracted and fibrosed. Regular mammograms were performed. Initially, there were no mammographic changes, but 4 years post-operatively, the breast parenchyma was contracted with new scattered benign calcifications. Over the next 10 years, the patient was seen regularly in clinic and her breast remained fibrosed with progressing calcification on mammogram (Fig. 1). In 2012, the calcification started to ulcerate through the skin extensively and with associated suppuration (Fig. 2). After much consideration, the patient underwent a completion mastectomy with a good post-operative recovery.

Don't miss it: a rare case of multifocal necrotizing fasciitis.

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