Abstract
Pyoderma gangrenosum (PG) is a rare neutrophilic disorder with an incidence rate of 3–10 cases per million per year, characterized by classically painful and aseptic ulcers, which may be associated with underlying systemic diseases. The pathergy reaction is seen in one-fourth of patients with PG. Accurate and timely diagnosis is crucial, as PG is known for its rapid progression. The management of PG is challenging and depends on its severity and rate of progression. An underlying systemic involvement should be sought even in spite of no symptoms. Herein, we report a case of giant pyoderma gangrenosum involving almost the entire left buttock with exceptionally raised c-ANCA levels, but no underlying systemic abnormality. The patient reported intense pain, rapid progression of the ulcers, an inability to perform daily activities, was significantly morbid and pathergy-positive. Aggressive and early management is required in cases such as this. A dramatic response was achieved with a combination of cyclosporine, dapsone, and methylprednisolone pulses.
Highlights
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by noninfectious ulcers, usually involving the lower extremities, with an estimated incidence rate of 3–10 cases per million per year [1]
PG is associated with underlying systemic diseases in around 50% of cases [2]
We report a case of giant pyoderma gangrenosum on an atypical site—the left buttock—with highly elevated c-ANCA levels, treated with multidrug therapy
Summary
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by noninfectious ulcers, usually involving the lower extremities, with an estimated incidence rate of 3–10 cases per million per year [1]. We report a case of giant pyoderma gangrenosum on an atypical site—the left buttock—with highly elevated c-ANCA levels, treated with multidrug therapy. The patient developed three small ulcers on the left buttock while on treatment with azathioprine, oral steroids, and minocycline for two consecutive months. An examination revealed a giant oval ulcer 23 × 17 cm in size covered with a necrotic brown crust on the left buttock Manipulation produced purulent exudate and oozing of the blood Another deep-seated ulcer, 2 × 3 cm in size and adjacent to the initial, was present medially. A deep skin biopsy from the edge of the ulcer revealed partially treated vasculitis, endothelial swelling, intravascular thrombi, and large neutrophilic aggregates in the subcutaneous tissue, consistent with the diagnosis of PG The levels of ESR, CRP, and c-ANCA (4.0 units/mL) have returned to normal
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