Staphylococcus aureus bacteremia and infective endocarditis in a patient with epidermolytic hyperkeratosis: A case report

Abstract

Background: Staphylococcus aureus bacteraemia (SAB) is among the leading causes of bacteraemia and infectious endocarditis. The frequency of infectious endocarditis (IE) among SAB patients ranges from 5% to 10%-12%. In adults, the characteristics of epidermolytic hyperkeratosis (EHK) include hyperkeratosis, erosions, and blisters. Patients with inflammatory skin diseases and some diseases involving the epidermis tend to exhibit a disturbed skin barrier and tend to have poor cell-mediated immunity.

Case summary: We describe a case of SAB and infective endocarditis in a 43-year-old male who presented with fever of unknown origin and skin diseases. After genetic tests, the skin disease was diagnosed as EHK.

Conclusion: A breached skin barrier secondary to EHK, coupled with inadequate sanitation, likely provided the opportunity for bacterial seeding, leading to IE and deep-seated abscess or organ abscess. EHK may be associated with skin infection and multiple risk factors for extracutaneous infections. Patients with EHK should be treated early to minimize their consequences. If patients with EHK present with prolonged fever of unknown origin, IE and organ abscesses should be ruled out, including metastatic spreads.

Keywords: Case report; Epidermolytic hyperkeratosis; Infective endocarditis; Staphylococcus aureus bacteremia.

Figure 1

Images of the skin lesions. Diffuse palmoplantar hyperkeratosis, erythema, and scales on the flexor surfaces of both arms and lower limbs. A: Palms; B: Opisthenar; C: Arms; D: Planta pedis; E: Dorsum of the foot.

Figure 2

Computed tomography. A and B: Cranial computed tomography showing multiple low-density shadows (blue arrow, A and B) in the brain; C and D: Abdominal enhanced CT scan showing multiple low-density shadows (green arrow, C and D) in the spleen, considered a splenic abscess with subcapsular effusion.

Figure 3

Transesophageal echocardiography. A and B: The anterior leaflet of the mitral valve was detected with a wart (white arrow) of a cord-like medium echoic substance about 10mm and the posterior leaflet was detected with the wart (white arrow) of a medium echoic substance about 7 mm × 7 mm; C and D: There was severe mitral regurgitation and the regurgitation bundle was distributed along the posterior leaflet of the mitral valve. There was mild tricuspid valve and the aortic valve regurgitation.

Figure 4

Histopathological, and genetic features of the patient. A and B: The c.539A>G mutation was detected in KRT1; C: The pathological examination indicated epidermal hyperkeratosis, acantholysis and lymphocytes infiltrating the superficial dermis around blood vessels and adjuncts (H&E stain, original magnification 100×); D: c.539A>G detection using Next Generation Sequencing and Sanger sequencing.