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A 57-year-old man with pemphigus vulgaris is admitted to the hospital following his arrival at the emergency room with fever, dysuria, and left flank pain.

His complex medical history also includes diabetes mellitus and chronic hydroureteronephrosis with an atrophic left kidney. In the recent past, he reports that he received treatment for deep vein thrombosis of the right lower limb complicated by pulmonary embolism.

On clinical examination at the time of his admission, he appears unwell. Palpation of his abdomen shows left costophrenic angle tenderness. However, otherwise it yields to gentle pressure, and is not distended or tender.

Laboratory tests reveal that the patient's white blood cell (WBC) count is within the normal range. The patient's urine culture reveals antibiotic-resistant Escherichia coli. He is diagnosed with pyelonephritis and hospitalized to receive intravenous antibiotic treatment.

When he is admitted, his current medications include enoxaparin sodium, metformin, gliclazide, ibuprofen, prednisone 100 mg, and azathioprine.

5 Days Later

After spending 5 days in the hospital, the patient begins to experience bloating of his abdomen, which he says is not relieved by defecation. He also reports being constipated. However, he notes that these symptoms are not causing him any pain.

Vital signs are unremarkable, and his WBC count remains within the normal range. Palpation of his abdomen confirms that it is distended, but continues to remain soft with no tender areas. Hypoactive bowel sounds are notable on auscultation.

The patient's symptoms are suspected to be due to his extended period of inactivity since being hospitalized. Clinicians prescribe laxatives and recommend that he spend some time walking the halls.

Several days thereafter, however, the patient's bloating and constipation have not improved.

Diagnosis and Treatment

A contrast-enhanced computed tomography (CT) scan of the abdomen and pelvic area is performed, which identifies perforated sigmoid diverticulitis with peri-sigmoidal abscesses (Figure).

These findings suggest a Hinchey III perforation in the mid-sigmoid colon, associated with purulent peritonitis resulting from diverticulitis of the colon. Subsequent to this finding, clinicians perform laparoscopic drainage of the purulent peritonitis and an abscess cavity. Laparoscopic drainage is completed without complications and the patient begins to feel better.

4 Days Post-Surgery

Then, 4 days following his surgery, the patient experiences more abdominal bloating, again without any accompanying pain. Another CT scan reveals a large pneumoperitoneum. Clinicians perform exploratory laparotomy and find perforation of a diverticula, in this case located in the distal sigmoid colon.

Case Follow-up

Clinicians perform sigmoid resection and colorectal mechanical anastomosis, as well as a diverting loop ileostomy to prevent an anastomotic leak. Notably, the patient's WBC count remains within the normal range throughout these episodes.

Closure of the ileostomy is performed 4 months later, and the patient has no further colonic perforations. However, clinicians report that he died a year later as a result of complications from fulminant pemphigus vulgaris.

Discussion

The clinicians presenting this ¹ of recurrent sigmoid colonic perforation highlight the fact that diverticular perforation can be asymptomatic in patients on immunosuppressive therapy. They encourage a high index of suspicion for bowel perforation in patients with abdominal symptoms being treated for skin diseases.

In particular, the authors note that immunosuppressive treatments may interfere with detection of inflammation and reduce symptoms, with the potential to delay diagnosis and definitive treatment. As in this case, the symptoms could be related to perforation of a viscus, or an acute surgical abdomen.

Colonic diverticulosis is typically asymptomatic – it involves multiple uninflamed diverticula (pouches) in the colon. However, it can cause mild abdominal pain, usually in the left lower quadrant, or in severe instances, generalized peritonitis and shock.²

Large bowel perforation is rare and can be associated with serious complications. The mortality rate in patients with colonic perforation ranges from 17% to almost 20%.^3,4 Perforation of the large bowel is readily diagnosed in the patient presenting with acute surgical abdomen, but may represent a diagnostic challenge when the clinical presentation is atypical.

Corticosteroid use has been associated with spontaneous ^5-7 that often cannot be contained in the early stages due to the immunosuppressive effect of the medication.⁸ Use of the disease-modifying antirheumatic drug (DMARD) methotrexate has been linked with a single isolated symptomatic case of spontaneous diverticular perforation in a patient with rheumatoid arthritis,⁹ the case authors report.

In the current case, the patient was at increased risk for this adverse effect due to receiving both corticosteroids and nonsteroidal anti-inflammatory drugs. The patient's unusual presentation may be the first reported case involving two consecutive episodes of perforated diverticula associated with abdominal distension, the authors suggested. They attributed the absence of signs of peritonitis or inflammation to the immunosuppressive effects of both steroids and the DMARD azathioprine.

Pemphigus vulgaris is an immune globulin G-mediated autoimmune disease marked by blisters and erosions of the skin and oral mucosa through acantholysis of the stratified squamous epithelium.¹⁰ It is typically treated with systemic corticosteroids, often with the addition of azathioprine and mycophenolate to reduce both steroid dose and the risk of steroid-related .¹¹

The authors urge clinicians treating immunosuppressed patients to perform investigations, including imaging, to prevent delay in definitive management. The clinicians also caution that patients taking immunosuppressive medications may have impaired wound healing. This may alter the approach to surgical management of colonic perforation in diverticulitis, including consideration of a more aggressive approach that includes Hartmann's procedure instead of laparoscopic lavage.

References

1. Saliba C, et al: Recurrent asymptomatic sigmoid diverticular perforation in a patient with pemphigus vulgaris on immunosuppressive therapy: A case report. Am J Case Rep 2019; 20: 735-738.

2. David J, Ozick LA: Diverticulitis. N Engl J Med 1995; 333(26): 1785-1786.

3. Kriwanek S, et al: Prognostic factors for survival in colonic perforation. Int J Colorectal Dis 1994; 9(3): 158-162.

4. Bielecki K, et al: Large bowel perforation: Morbidity and mortality. Tech Coloproctol 2002; 6(3): 177-182.

5. Weiner HL, et al: Sigmoid diverticular perforation in neurosurgical patients receiving high-dose corticosteroids. Neurosurgery 1993; 33(1): 40-43.

6. Kaya B, et al: Steroid-induced sigmoid diverticular perforation in a patient with temporal arteritis: A rare clinical pathology. Clin Med Insights Pathol 2012; 5: 11-14.

7. Mpofu S, et al: Steroids, non-steroidal anti-inflammatory drugs, and sigmoid diverticular abscess perforation in rheumatic conditions. Ann Rheum Dis 2004; 63(5): 588-590.

8. Morris CR, et al: Epidemiology of perforated colonic diverticular disease. Postgrad Med J 2002; 78(925): 654-658.

9. Chang I, et al: A case of diverticular perforation in a young patient with rheumatoid arthritis on methotrexate. Case Rep Med 2015; 2015: 617268.

10. Hammers CM, Stanley JR: Mechanisms of disease: Pemphigus and bullous pemphigoid. Annu Rev Pathol Mech 2016; 11: 175-197.

11. Yasir M, Sonthalia S: Corticosteroid Adverse Effects. [Updated 2019 Mar 24]. In: StatPearls, Treasure Island (FL): StatPearls Publishing; 2019 Jan, Accessed May 26 2019 at:

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