Fever + rash, the elderly have common diseases in children, be careful that cancer is at work!

*Read only for medical professionals.
Why is a disease prevalent in children suddenly appearing in elderly patients? In a recent case report published in Rheumatology [1], two unusual cases were reported, both in elderly patients, but with symptoms consistent with a disease often seen in children
.

What is the specific situation? Let's see together! The fog is heavy - the case is on the scene ■ Case 1: A 59-year-old male was admitted to the hospital because of "fever and rash for 9 days"
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The patient had persistent fever ≥39°C with rash in the past 9 days
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Initial physical examination revealed a pleomorphic rash on the hands, oral rash, and strawberry tongue, but no conjunctivitis or cervical lymphadenopathy
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There was a history of prostate cancer surgery and recurrence
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Four days after admission, the patient presented with peeling hands (Figure 1) and abdominal pain
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 Figure 1: Auxiliary examination results for the patient's hand peeling signs: abdominal CT scan: suggestive of non-biliary pancreatitis (Balthazar grade C), ileitis, right-sided colitis, severe gastric mucosal thickening with gastric adenopathy (Figure 2B).
)
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CT scan of the great arteries: left subclavian arteritis (Fig.
2C)
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Echocardiography and coronary CT angiography: No cardiac involvement was found
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Gastroscopy: gastric mucosal infiltration and hypertrophy, pathological anatomy results confirmed gastric adenocarcinoma
.

Laboratory findings: elevated C-reactive protein (325 mg/L); neutrophilic leukocytosis (40 g/mm3); thrombocytosis; hypoalbuminemia (18 g/L); hepatolysis with Hyperbilirubinemia and elevated lipase levels (1300 UI/L); blood cultures were negative
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 Figure 2: Imaging pictures of this patient What other diagnoses do you think might be possible in addition to the diagnoses given above for this case? ■ Case 2: A 77-year-old male, who went to the doctor because of "fever with rash for 7 days"
.

 The patient had persistent high fever for 7 days with a body temperature of ≥38.
5°C; he had multiple rashes all over the body, including a pleomorphic rash with palmoplantar involvement (Fig.
3D) and an oral rash (Fig.
3E)
.

 Figure 3: Manifestation of the patient's rash.
The patient had a history of double-heterozygous hemochromatosis (C282Y and H63D) and atrial fibrillation
.

 Physical examination revealed bilateral conjunctivitis and cervical lymphadenopathy, as well as signs of hand ischemia with distal necrosis, in addition to a pleomorphic rash with palmoplantar involvement and an oral rash
.

 Figure 4: Signs of hand ischemia with distal necrosis in the patient.
Ancillary findings: Vascular Doppler ultrasonography: normal; laboratory tests: elevated C-reactive protein level (236 mg/L), neutrophilic leukocytosis (14 g/mm3), sterile leukocytouria; blood culture: negative; skin biopsy: signs of leukocytoclastic vasculitis; esophageal CT scan: esophagus thickening, biopsy confirmed esophageal squamous cell carcinoma
.

Seeing such a patient, can you give any other diagnosis? Clearing the Clouds and Seeing the Sun - Case Analysis ■ What else is there to diagnose? First, based on gastrointestinal symptoms and pathological results, it was clear that both patients had cancer, the first patient had gastric cancer and the second patient had esophageal cancer
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 However, both patients also had manifestations other than the typical symptoms of gastrointestinal tumors, such as: high fever, rash, diffuse congestion of the oral mucosa, conjunctivitis, and nonsuppurative lymphadenopathy
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and many more! This series of symptoms seems a little familiar.
.
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That's right! Isn't this the main clinical manifestation of Kawasaki disease (KD) common in children! KD is an acute self-limited vasculitis of unknown etiology, mainly involving small and medium arteries
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Because its clinical manifestations are mainly fever, conjunctivitis, rash, lymphadenopathy, etc.
, it is also known as mucocutaneous lymph node syndrome
.

 KD occurs mainly in children before the age of 5 years, and adult-onset Kawasaki disease (AKD) is rare
.

A review of AKD patients showed that the average age of AKD was 30 years [2]
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 "Chinese Journal of Pediatrics" published the "Expert Consensus on the Diagnosis and Acute Treatment of Kawasaki Disease" in January 2022 [3], updating the clinical diagnostic criteria and classification of KD: KD is a clinical syndrome, and the diagnosis mainly depends on clinical manifestations combined with laboratory Check and rule out other diseases
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KD includes complete Kawasaki disease (CKD) and incomplete Kawasaki disease (IKD)
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 CKD is manifested as fever and has at least 4 main clinical features of the following 5 items: (1) bilateral bulbar conjunctival hyperemia; (2) changes in the lips and oral cavity: dry red lips, strawberry tongue, diffuse oropharyngeal mucosa Congestion; (3) rash, including the red and swollen scars that appeared alone; (4) changes in peripheral circulation of the extremities: redness and swelling of the hands and feet in the acute stage, and periungual peeling in the recovery stage; (5) non-suppurative cervical lymphadenopathy
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 IKD: Fever ≥ 5 days, but the main clinical features are less than 4, and whether it is IKD is assessed according to the process shown in the figure below
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Figure 5: Diagnostic flow chart of IKD According to the diagnostic criteria, it can be seen that the patient in case 1 is IKD (fever, 3 main symptoms and 4 laboratory criteria), and the patient in case 2 is CKD (fever and 5 main symptoms) )
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■ Tumor with KD? What combination is this? Yes, this is not a fabricated case, but a real old man with tumor combined with KD
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But why KD-like symptoms commonly seen in children appear in older adults with tumors is unclear, as the pathophysiology of KD remains unanswered
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 However, the coexistence of gastrointestinal cancer and KD-like vasculitis in this case report suggests a potential pathophysiological link between the two
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There is previous evidence that the "gut-vascular" axis is involved in the pathogenesis of KD through the production and deposition of IgA in the intestinal mucosa in the case of intestinal barrier defects [4]
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Therefore, we can hypothesize that in the two cases reported here, the tumor may have destroyed the digestive mucosa, leading to an immune response and systemic vasculitis
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■ Treatment According to the KD treatment strategy, both patients received a single dose of intravenous immunoglobulin (IVIG, 2 g/kg) and oral aspirin (100 mg/kg/d used in case 1 and 100 mg/kg/d in case 2).
The dose used was 50 mg/kg/d)
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 Since the heart of the patient in case 1 was not involved, the treatment effect was good
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Abdominal pain and fever subsided over the next few days, and ileitis/colitis on CT scan returned to normal
.

For his gastric adenocarcinoma, chemotherapy and gastrectomy were given
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Five months later, bone metastases were found
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 In case 2, the patient did not improve after 10 days of treatment, and the new IVIG dose (1 g/kg) and corticosteroid treatment (1 mg/kg) after 3 weeks did not improve symptoms
.

Subsequent examination revealed myocarditis, global hypokinesis of the left ventricle, decreased ejection fraction, and elevated troponin level (663 µg/L)
.

The patient eventually developed cardiogenic shock and died
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Shifting the Stars - Case Rewards This case report is the first to discuss two cases of AKD-like vasculitis as a paraneoplastic syndrome in the context of gastrointestinal cancer
.

In addition, the patient in case one had signs of left subclavian arteritis on CT scan, which is rare in AKD
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The patient in case two presented with symptoms of hand ischemia, an extremely rare complication reported in young infants with KD, and this patient was refractory to IVIG therapy, which is atypical in AKD disease
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 In short, do not underestimate KD, this is not an exclusive disease of children! If the main symptoms of KD are found in elderly patients, beware of paraneoplastic syndrome! References [1] Hippolyte Lequain, Jean Baptiste Fraison, et al.
Adult-onset Kawasaki-like disease as a paraneoplastic syndrome.
Rheumatology [J], 2022 Mar 18.
[2] Fraison JB, Sève P, Dauphin C, et al .
Kawasaki disease in adults: Observations in France and literature review.
Autoimmun Rev.
2016 Mar;15(3):242-9.
doi: 10.
1016/j.
autrev.
2015.
11.
010.
Epub 2015 Nov 26.
PMID: 26631821.
[ 3] Cardiovascular Group of Pediatrics Branch of Chinese Medical Association, Rheumatology Group of Pediatrics Branch of Chinese Medical Association, Immunology Group of Pediatrics Branch of Chinese Medical Association, Editorial Board of Chinese Journal of Pediatrics.
Expert consensus on diagnosis and acute treatment of Kawasaki disease[J] ].
Chinese Journal of Pediatrics, 2022, 60(01):6-13.
[4]Noval Rivas M, Arditi M.
Kawasaki disease: pathophysiology and insights from mouse models.
Nat Rev Rheumatol.
2020 Jul;16(7):391 -405.
doi: 10.
1038/s41584-020-0426-0.
Epub 2020 May 26.
PMID: 32457494; PMCID: PMC7250272.