Abstract

A 70-year-old lady with diabetes and monoclonal gammopathy underwent pacemaker implant for 2:1 atrioventricular block. Within 7 days, a painful, infiltrating, necrotic lesion involved the implant area. Biopsy was compatible with pyoderma gangrenosum and corticosteroid treatment led to healing in 3 weeks.

Introduction

Pyoderma gangrenosum (PG) is an inflammatory process of unknown aetiology that can complicate minor trauma, including surgical incisions. We report a case of PG after permanent pacemaker implantation that was controlled with corticosteroids.

Case report

A 79-year-old woman with a history of hypertension, diabetes, and monoclonal gammopathy (IgG lambda), without diagnostic criteria for multiple myeloma, underwent a permanent VDD pacemaker implant for 2:1 AV block associated with heart failure. The electrode was advanced through a left subclavian puncture and the generator was placed under the anterior pectoral fascia. Immediate course was uneventful and she was discharged the following day, but fever (38°C) and local pain and swelling developed thereafter. On the seventh day after implant, there was an area of inflammation with deep infiltration and cutaneous blistering, 12 cm in diameter, surrounding the incision. A superficial culture grew Staph epididymis, and cloxacyclin was started. In 3 days, the inflammatory area became dark and coarsely granular in the centre (Figure 1B), and a biopsy showed massive neutrophilic infiltration and extensive necrosis compatible with PG. Systemic corticosteroid therapy was started with 60 mg of prednisone daily. Pain and inflammation subsided rapidly and the lesion healed completely in 3 weeks. Concomitantly, the patient developed progressive heart failure and a severe nephrotic syndrome that led to her death 1 month after admission. Cardiac and renal amyloidosis was suspected, but no biopsy was performed and a post-mortem examination was not authorized.

Figure 1

(A) Erythematous, infiltrated plaque with beginning central necrosis and (B) evolving lesion 2 days later. Necrotic ulcer with coarse granulation tissue and bluish borders forming circular segments.

Figure 1

(A) Erythematous, infiltrated plaque with beginning central necrosis and (B) evolving lesion 2 days later. Necrotic ulcer with coarse granulation tissue and bluish borders forming circular segments.

Discussion

PG is a rare ulcerative process of unknown aetiology, attributed to an immunological reaction, related to a dysfunction of neutrophils.1 It can involve the skin or internal organs, and in 25% of cases, it is triggered by minor skin injury2,3 or surgery, such as pacemaker implantation,4,5 a phenomenon alluded to as pathergy. It is usually observed between age 20 and 50, but it can occur at any age. In >50% of cases, it is associated with Crohn's disease or ulcerative colitis, rheumatoid arthritis, or haematological diseases, such as leukaemia, or myelofibrosis of monoclonal gammopathy, as in our case.2 PG starts as a painful erythematous plaque, a nodule, blister, or pustule, evolving rapidly to central necrosis, with bluish circular edges and granulation tissue with the appearance of ‘hamburger meat.’ Healing occurs from the edges, leaving atrophic, cribriform, often-pigmented scars. The diagnosis is made by the clinical and pathological correlation, because the biopsy is non-specific, but it allows ruling out of other processes.1 Treatment with prednisone, immunosuppressant agents, or necrosis factor inhibitors may allow healing without pacemaker explant, as in our case.6 Unfortunately, our patient died of comorbid conditions, despite healing of the PG lesions.

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Supplementary data