Scrotal and penile edema in a patient with incomplete kawasaki disease: a case report and brief literature review

Abstract

Background

Kawasaki disease (KD) is a medium artery vasculitis that predominantly affects children under age 5. Prompt diagnosis and treatment with IVIG and moderate dose aspirin is required to prevent the formation of coronary artery aneurysms. While scrotal edema and erythema have been seen in KD, here we present a distinctive case of incomplete Kawasaki with these features as well as penile edema.

Case presentation

A 2-year-old, unvaccinated, African American male presented with 4 days of fever, bilateral limbic sparing conjunctivitis, a papular rash, unilateral shotty cervical lymphadenopathy, mild right-hand edema, and scrotal and penile edema and erythema. His labs were significant for sterile pyuria, elevated ALT, anemia for age, and hypoalbuminemia. He was diagnosed with incomplete Kawasaki disease and was treated with IVIG and moderate dose aspirin. Echocardiogram was negative for coronary aneurysms. His symptoms resolved and he was discharged home with low dose aspirin. At his 2-week follow up, he remained well-appearing with no refractory Kawasaki symptoms.

Conclusion

This is a unique case of penile edema in KD which to our knowledge has not been previously reported in literature. An understanding of genitourinary symptoms in Kawasaki disease can help timely diagnosis and treatment of the disease.

Background

Kawasaki disease (KD) is a common, medium artery vasculitis that predominantly affects children under the age of 5 [1]. It is usually self-limiting, although if left untreated, can lead to coronary artery aneurysms, myocardial infarct, heart failure, and arrhythmias [1]. Kawasaki is diagnosed in patients with a fever lasting longer than 5 days and in those that have 4 out of the following criteria: conjunctivitis, rash, mucocutaneous changes, cervical lymphadenopathy, and peripheral extremity changes including erythema or edema [1]. In patients with a prolonged fever and 2–3 of the clinical criteria, the presence of 3 supplemental lab criteria can be used to diagnose incomplete KD [2]. The supplemental lab criteria include anemia for age, platelet count over 450,000 after day 7 of disease, albumin less than 3 g/dL, elevated ALT, leukocytosis over 15,000/mm³, and sterile pyuria [2]. Here, we present a case of incomplete KD accompanied by penile edema, an uncommon finding in KD patients.

Case description

A 2-year-old, unvaccinated, previously healthy African American male presented with 4 days of fever and rash. He was initially seen at the ER on day 2 of illness for intermittent fevers with T_max 38.9 °C and a papular rash that started on his torso and back before spreading into his diaper region. The patient tested negative for strep throat and was discharged home with a diagnosis of viral exanthem. On day 4 of illness, the patient returned to the ER with persistent fevers, worsening skin rash, and swelling of his penis and scrotum. He was normotensive, febrile (T 38.7 °C), tachycardic (180 bpm) and tachypneic (44 respiratory rate) with oxygen saturation of 98% on room air. Lab work was significant for elevated C-reactive protein (146 mg/L) and erythrocyte sedimentation rate (70 mm/hr), hyponatremia (133 mMol/L), direct hyperbilirubinemia (total 4.25 mg/dL, direct 1.51 mg/dL), and elevated liver enzymes (AST 95 units/liter, ALT 228 units/liter). CBC was unremarkable and his urinalysis showed sterile pyuria. EBV and hepatitis panels were negative, RVP was sent and a blood and urine culture was drawn. Abdominal ultrasound was negative for hepatic abnormalities and showed a distended gallbladder but without stones or sludge. Due to early concern for staphylococcal scalded skin syndrome and sepsis, the patient was started on IV antibiotics (ceftriaxone and clindamycin) and IV fluids and was admitted to the infectious disease service for additional workup and management.

On physical examination, the patient had bilateral conjunctivitis with limbic sparing, shotty unilateral cervical lymphadenopathy, and a flesh-colored papular rash on the chest, back, and posterior right leg. The patient’s penis and scrotum were swollen, erythematous, and tender to touch. Scrotal ultrasound showed scrotal wall edema and hyperemia (Fig. 1). Urology was consulted and also noted that the patient’s penis was edematous with most of the edema present along the preputial cuff and recommended using a barrier ointment and doing serial exams. Echocardiogram came back negative for coronary artery aneurysm. On day 2 of admission, his respiratory viral panel came back positive for rhino/enterovirus and his blood and urine cultures came back negative. His scrotal edema was also observed to have resolved and his penile swelling had improved, but did not resolve. He continued to be febrile, his blood albumin level decreased to 3.0 g/dL, and he became anemic for his age (Hb 10.4 gm/dL). He was diagnosed with incomplete KD due to the presence of 3 clinical criteria and 4 supplemental lab criteria. His antibiotics were stopped and he was started on IVIG (2 g/kg over 12 h) and moderate dose aspirin (30 mg/kg divided q6H). Over the next few days, the patient’s rash and conjunctivitis improved. He defervesced on day 4 of admission, and his penile swelling continued to improve. On day 5 of admission, he was transitioned to low dose aspirin (81 mg daily) and discharged home.

Fig. 1

Scrotal ultrasound showing normal testes (black triangle) with scrotal wall edema and hyperemia (gray triangle)

Follow up approximately 2 weeks after discharge revealed that the patient had made a complete recovery. His repeat echocardiogram was negative for coronary artery dilation and his physical exam was negative for periungual desquamation. He was continued on aspirin until his next echocardiogram. Follow up at 2 months showed a normal echocardiogram and his physical exam remained negative for periungual desquamation.

Discussion

KD is an acute self-limiting vasculitis with unknown etiology. While it is easy to diagnose if all clinical criteria are present, in the absence of such features, many patients may end up getting misdiagnosed with sepsis and treated unnecessarily with antibiotics. A delay in diagnosis could also lead to increased risk of coronary aneurysms and other complications. Our patient presented with persistent fevers, skin rash, unilateral cervical lymphadenopathy and bilateral conjunctivitis supporting a diagnosis of underlying vasculitis. An atypical finding in this case was that he also had scrotal and penile edema.

KD has been reported to be associated with an array of genitourinary findings. Some reports have documented scrotal edema that spared the penis in patients with KD [3, 4]. Our literature search did not reveal any previously reported cases of penile edema in KD. Urogenital manifestations of other vasculitides were reviewed to identify similarities with this case. In children, Henoch-Schoenlein purpura (HSP), a small vessel vasculitis, has been associated with scrotal edema, epididymitis, orchitis, and spermatic cord thrombosis [5,6,7] and in rare cases, has also been associated with penile edema, erythema, and penile pain [8]. Although not classically seen in vasculitides, clinicians should still keep KD and HSP in the differential when considering patients with scrotal and penile edema.

Diagnosis of KD is challenging especially when a patient presents with atypical or nuanced features. Considering our patient’s atypical presentation of penile edema, infectious work-up was done and was negative. This signified that his edema most likely stemmed from his vasculitis. From what we have seen in the literature, a possible mechanism for this is that the strong inflammatory response from KD produces radical oxygen and nitrogen species that damage the endothelial lining [9] and in a highly vascular structure like the penis, this can cause edema due to the unregulated release of fluid into surrounding tissues. This leakage is likely compounded by the systemic release of inflammatory cytokines that occurs during KD [10]. This is only a proposed mechanism and further research should be done to fully elucidate why this might occur in some patients versus others.

Conclusion

KD is an acute systemic vasculitis that can lead to coronary artery and other medium vessel aneurysms. Because time to treatment can influence patient outcomes, it is important to prevent delays in the diagnosis of incomplete KD. Penile edema is a rare presentation of other vasculitides in children such as HSP and here, we present an atypical case of it in KD.