A 79-year-old woman with hypertension, ischemic heart disease, and Hashimoto's disease sustained a closed fracture of the left distal radius due to a fall from patient's height. The patient underwent closed reduction and immobilization in a plaster cast. Because of secondary displacement, eight weeks later, patient received open reduction and stabilization with a volar titanium locking plate and screws. After surgery, the patient suffered from severe pain and edema, but ultrasound showed no pathology related to the performed operation, and the symptoms relieved spontaneously.
After a 2.5-year asymptomatic period, the patient reported pain and edema of patient's left wrist. The radiograph showed the loosening of one screw, causing conflict with soft tissues. We removed the implant, but we also found serous masses penetrating the surrounding tendon sheaths. Microbiological cultures revealed no pathogens; tuberculosis was excluded as well. Due to suspected infection, the patient empirically received clindamycin and ciprofloxacin.
One month after the implant removal, the surgical wound presented as ulceration with fistulae and purulent discharge (Fig. 1); radiographs revealed osteolysis within the styloid process of the radius. Despite many negative microbiological cultures, we still suspected infection as the most probable cause. We performed debridement of the lesion with application of local antibiotic carrier gentamicin beads and collagen gentamicin sponge with the addition of 1 g of cefazolin and 1 g of vancomycin. Negative pressure wound therapy (NPWT) to improve wound healing followed the surgery. After two weeks, wound healing was satisfactory.
After another month, radiographs showed a pathologic fracture in the site of the previously treated osteolytic bone defect and screw canals. We performed external fixation with wrist canal decompression and local administration of gentamicin beads again. The wound healing was slow and microbiological cultures revealed Enterococcus faecalis. Targeted antimicrobial therapy with levofloxacin resulted in only partial improvement. As new purulent skin lesions appeared, we took skin and bone biopsies (Fig. 2).
Histopathology of the bone was unspecific, but excessive numbers of neutrophils and plasmacytes were found in the skin. Radiographs showed a progressing deformation of the wrist and osteomyelitis (Fig. 3 A, B). After exclusion of plasmacytoma and other hematologic conditions, PG was diagnosed.
The introduction of immunosuppressive treatment with prednisone and CsA gave excellent clinical effects. Unfortunately, every attempt to reduce the prednisone dose below 20 mg/day led to the lesion's recurrence despite concurrent CsA treatment with an adequate trough level of 100-130 ng/ml. Given the poor patient tolerance of the treatment and two episodes of superinfection, the immunologic treatment was not continuous, leading to a massive PG relapse (Fig. 4 A, B).
Severe pain, deformation of the extremity, and function alteration were the reasons for amputation of the proximal 1/3 of the forearm. The immunosuppressive treatment was discontinued after surgery because of the risk of infection. The wound healing was primarily good, but PG lesions relapsed in the stump after several weeks. Magnetic resonance showed fistulae and edema in soft tissues and osteomyelitis in forearm bones, but the culture from the wound was negative. Immunosuppressive treatment was started again, with an initially good response. Unfortunately, a massive relapse of ulcers occurred after decreasing the prednisone dose below 10 mg/day.
The next half of a year of local and systemic treatment did not succeed, which ultimately led to exarticulation in the scapulohumeral joint. At this point in time, 6 years had already passed since the primary fracture. The wound healing was good, but immediately after the surgery, the patient was diagnosed with COVID-19. High-resolution computed tomography scans showed that the viral inflammation affected 90% of both lungs. The patient was in a severe condition and required 6 mg/day of dexamethasone and high-flow oxygen therapy (40 l/min 100% O2). Five days later, when patient's condition improved, oxygen supplementation was limited and ultimately ended after 10 days.
After two weeks, the patient was discharged home on prednisone 30 mg/day. The prednisone dose was gradually reduced and withdrawn after 8 months.
There was a relapse of PG one year after disarticulation - a purulent ulcer with morphology typical for PG in the postoperative scar. After a return to prednisone therapy, the ulcer healed completely. Now, every attempt to reduce prednisone dose below 30 mg/day results in a relapse of PG lesions, making the patient GC-dependent. Currently, the 85-year-old patient is suffering from iatrogenic Cushing syndrome, with kidney failure and fatigue as the most prominent symptoms. We are applying local GCs to avoid increasing the dose of systemic GCs.
Ethical standards: The written consent of the patient for publishing the case report was obtained.