Acute appendicitis complicated with necrotizing fasciitis in a patient with adult-onset Still's disease

Abstract

Rationale:Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, spiking fever, arthralgia/ arthritis, and lymphadenopathy. AOSD sometimes was fatal when it is complicated by macrophage activation syndrome (MAS) or hemophagocytic lymphohistiocytosis (HLH). Nonetheless, the literature provides no recommendations for treatment of AOSD patients with severe sepsis.Patient concerns:A previously healthy 65-year-old man with history of AOSD was referred to our hospital for persistent right lower quadrant abdominal pain for 2 days. One week later, an abdominal wall abscess and hematoma developed by extravasation from the inferior epigastric vessels, complicated by necrotizing fasciitis of the right thigh and groin region. To our best knowledge, this case was the first reported case of a perforated appendix complicated with necrotizing fasciitis in a patient with AOSD.Diagnoses:The patient was diagnosed as acute appendicitis complicated with necrotizing fasciitis and abdominal wall abscess.Interventions:This case received intravenous tigecycline injection and daily 10 mg prednisolone initially, and shifted to daily intravenous hydrocortisone 200 mg for suspected MAS or HLH. This patient underwent surgical intervention and debridement for necrotizing fasciitis.Outcomes:The patient's symptoms progressed worse rapidly. He died from cytomegalovirus viremia and bacterial necrotizing fasciitis complicated by septic shock.Lessons:(1) The steroid dose was difficult to titrate when AOSD complicated by sepsis. The differential diagnosis from MAS/HLH with bacterial/viral infection related severe sepsis was difficult but critical for decision making from clinicians and rheumatologists. (2) The conservative treatment with antibiotics for perforated appendix is safe but has a higher failure rate in immunocomprised patients such as systemic lupus erythematosus and AOSD. Early surgical intervention might contribute to better outcome. (3) The abdominal wall abscess can be spread from intra-abdominal lesion through the inferior epigastric vessels which were as weak points of abdominal wall. Imaging examinations contribute to acute diagnosis and help surgeons perform surgical interventions to prevent morbidity and mortality.

Nonoperative management of appendicitis in children

“…Prior to the well referenced description of "perforating inflammation of the vermiform appendix" by Dr. Reginald Fitz in 1886, surgeons had been extirpating the appendix and discovering the evolution of appendicitis as a clinical disease process (9). The first surgeon on record, Claudius Amyand, eponymous for Amyand's hernia (an appendix identified within a hernia sac) identified an inflamed appendix in an inguinal hernia of a young boy in 1736 (10).…”

Case report of open appendectomy in treating acute perforated appendicitis with necrotizing fasciitis of the abdominal wall: A rare complication of a common disease

“…8 Although wide‐spectrum antibiotic treatment is thought to be a safe management plan in treating appendicitis, it has a 7% failure rate, most commonly seen in immunosuppressed patients who mainly use corticosteroids. 9 …”

“…The disease is mainly observed in immunodeficient patients, in whom the mortality rate can be up to 30%. 4 , 8 , 9 Adult NF occurs more frequently in the abdomen, perineum, and limbs, but in children, especially infants, it usually occurs in the trunk. 13 NF in infants after acute appendicitis is rare and fatal, but exceptions have also been reported, which require prompt diagnosis with aggressive medical and surgical interventions.…”

“…8 Although wide-spectrum antibiotic treatment is thought to be a safe management plan in treating appendicitis, it has a 7% failure rate, most commonly seen in immunosuppressed patients who mainly use corticosteroids. 9 Necrotizing fasciitis (NF) causes a series of deadly infections that include inflammation followed by necrosis of the skin, subcutaneous fat, and fascia, which may also be associated with inflammation of the underlying muscle and may even lead to systemic sepsis. 10,11 It can occur with redness, swelling, and pain.…”

A Rare Complication of Perforated Appendicitis: A Case of Necrotizing Fasciitis

“…Moreover, it alone carries a 13.6% mortality [1]. On the other hand, a complicated perorated appendix with necrotizing fasciitis has a higher mortality rate, reaching 30-38% [8]. Early aggressive surgical debridement after diagnosis must take place to avoid systemic sepsis and enhance patient survival [1].…”