Multisystem involvement with ischemic complications in a child with STEC-HUS: a case of gangrene.

BACKGROUND

Skin involvement in haemolytic uremic syndrome (HUS) raises suspicion for atypical HUS; here, we report a child with gangrene and confirmed STEC-HUS.

CASE-DIAGNOSIS/TREATMENT: A 3-year-old boy with aggressive HUS presented with leukocytes 33,100/mm3, haemoglobin 7.8 g/dL, platelets 93,000/mm3, LDH 6020 IU/L, creatinine 2.4 mg/dL, sodium 124 mEq/L, albumin 1.9 g/dL, C-reactive protein 94 mg/L. He required peritoneal dialysis, mechanical ventilation for seizures, and milrinone for cardiac dysfunction. On day seven, ischemic lesions on two fingertips developed, prompting plasma infusions. Skin biopsy confirmed thrombotic microangiopathy (TMA). Further investigations confirmed STEC infection (anti-LPS IgM positive) and excluded aHUS and other TMA causes, allowing plasma therapy discontinuation and avoidance of eculizumab. After 21 days of dialysis and 13 days of mechanical ventilation, the patient was discharged. Two months later, fingertip auto-amputation occurred.

CONCLUSIONS

This case highlights the importance of differentiating STEC-HUS from aHUS when skin involvement is present, given the major therapeutic and prognostic implications.