1Samy Zakout, 1Toufik Tabbara
1Medcare Hospital
Background:
Pyoderma gangrenosum (PG) is an uncommon inflammatory and ulcerative skin disorder characterised histopathologically by the accumulation of neutrophils in the skin. The most common presentation of PG is the rapid development of one or more painful, purulent ulcer with undermined borders on sites of normal or traumatised skin.
Method(s):
A 19-year-old female patient with an unremarkable past medical history was referred last March to rheumatology by her dermatologist because of the suspicion of PG. She first received broad-spectrum antibiotics by her dermatologist for those ulcers and when there was no response to them, a biopsy was taken and a referral to rheumatology was recommended.
At her first presentation in the rheumatology clinic, she gave a 10-day history of rapidly evolving deep and painful ulcers on both legs. On further systemic inquiry, she happens to have had joint pain and swelling in both hands that predated the occurrence of ulcers by two months for which no medical advice had been sought. There was history of weight loss and fatigue, but the patient had no other symptoms otherwise.
On examination, she had symmetrical small joint synovitis in both hands, and a total of 4 purulent leg ulcers with undermined borders on both legs measuring up to 7cm in the biggest diameter.
As PG was strongly suspected, the patient was first commenced on a moderate dose of prednisolone at 20mg daily and a full set of blood tests was arranged.
Result(s):
When seen at the rheumatology clinic two days later, she reported some improvement in her legs pain and her blood tests confirmed an increased inflammatory response with a C-reactive protein of 46 and erythrocyte sedimentation rate of 50. She had neutrophilic leucocytosis. Her skin biopsy result then confirmed this diagnosis of PG. Her antinuclear antibodies, rheumatoid factor, cyclic citrullinated peptide and antineutrophil cytoplasmic antibodies were all negative.
At this stage, the patient was commenced on a glucocorticoid regimen of 0.75mg/kg/day (35mg) and referred to general surgery for optimal wound care.
At her subsequent reviews, she was showing symptoms and signs of improvement in her joints and skin. Her synovitis completely resolved. Her ulcers in particular were healing promptly and decreasing in size. In order to allow glucocorticoid tapering without disease recurrence, she was commenced on ciclosporin 100mg daily with regular kidney function and blood pressure monitoring. Of note, her leg ulcers completely healed up over the space of two months leaving only post-inflammatory hyperpigmentation. Her inflammatory response completely normalised. The patient is currently on prednisolone 5mg daily and has been on anti-TNF therapy with adalimumab for two months as her blood pressure started to rise. Of note, methotrexate was avoided initially as her liver enzymes were elevated during the acute stage.
Conclusion(s):
Despite the aggressive nature of PG and the potential difficulty in treating it, this patient showed a remarkable and swift response to standard immunosuppression without the need for intravenous immunoglobulins. Moreover, this case is being reported as it would rather be unusual for seronegative rheumatoid arthritis to present first with PG in an otherwise healthy patient. On the other hand, it can also be concluded that rheumatoid arthritis, to which the patient fully satisfied the relevant classification criteria, would have been the main reason behind this PG taking into account that no alternative causes, such as inflammatory bowel disease, were suggested when her calprotectin was negative. Also, the speed of ulcers progression over only 10 days made another point worth reporting.