Pyoderma gangrenosum in ulcerative colitis: considerations for an earl
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《英国医生杂志》
1 Department of Ophthalmology, Broomfield Hospital, Chelmsford, CM1 7ET, 2 Department of Plastic and Reconstructive Surgery, "G. Gennimatas" 6th IKA Oncological Hospital, 11473, Athens, Greece
Correspondence to: K I Papageorgiou papageorgiouk@doctors.org.uk
Pyoderma gangrenosum is a poorly understood destructive cutaneous disorder, characterised by progressive painful ulceration.1 Accurate epidemiological data are missing, but in half of cases there is an associated underlying disease, most commonly inflammatory bowel disease, rheumatological and haematological disorders.2 3 As pyoderma gangrenosum is not commonly encountered by clinicians, the diagnosis of such lesions is not always straightforward. We report the case of a man with ulcerative colitis, who presented with a non-healing ulcer of traumatic origin unresponsive to conservative and surgical management. This case emphasises the importance of detailed history taking and the consideration of pyoderma gangrenosum as a differential of such lesions in patients with a background of related systemic disease.
Case report
A 49 year old man was referred to the department of plastic surgery with a two week history of a post-traumatic, painful, and expanding ulcerated area on the anteromedial aspect of the left lower limb. The patient had a past medical history of ulcerative colitis, which he reported to be in remission for several years and he was currently not on any medication, including steroids. On initial presentation, the lesion was well demarcated measuring 6 x 4 cm with slightly undermined edges. Examination was otherwise unremarkable and conservative management was initiated. A week later, the lesion expanded to 12 x 8 cm, after which we debrided the wound and covered it with a split thickness skin graft. The postoperative course was uneventful, and the patient was discharged.
Six months later, the patient returned with a new ulcerated lesion, adjacent to the previous skin graft. Again, it was reported as post-traumatic. The lesion measured 5 x 8 cm (figure) and was indurated with violaceous undermined edges, atypical of a traumatic ulcer. Cultures were sterile and blood tests showed a normocytic anaemia and raised erythrocyte sedimentation rate.
Ulcerated lesion measuring 5 x 8 cm, on the middle third of the anterolateral aspect of the lower left leg, showing well demarcated violaceous edges
Cytological examination of scrapings from the ulcer base showed inflammatory features, and there was no bony involvement on radiography. The atypical features of the lesion and the abnormality of the blood tests prompted us to revisit the history. In depth questioning found that the ulcerative colitis was not inactive as reported and the patient was in fact experiencing bleeding from the rectum and had neglected to take his recently prescribed mesalazine and prednisolone.
Based on these findings, we considered the possibility of pyoderma gangrenosum as a differential, and we took an incisional biopsy of the lesion. Histopathology showed oedema, lymphocytic vasculitis, and neutrophilic infiltrates—features consistent with pyoderma gangrenosum.
The patient promptly started a course of 40 mg of oral prednisolone once daily and topical clobetasol. After a month, the ulcer had almost completely healed. In retrospect, the initial rapidly expanding ulcer that was treated with a skin graft was probably undiagnosed pyoderma gangrenosum.
Discussion
The diagnosis of pyoderma gangrenosum is based primarily on clinical presentation and course; histology can also be of value. The causes of pyoderma gangrenosum are unknown, but autoimmune mechanisms are probably implicated.4 Inflammatory bowel disease is the most common underlying disorder and is found in 10-15% of pyoderma gangrenosum cases.5 Of those with active ulcerative colitis, 7.1% may develop ulcerative colitis as an extraintestinal manifestation.6 Occurrence at sites of trauma or surgery is rare but well documented.7
Pyoderma gangrenosum most commonly occurs in adults aged 30-50, on the lower extremities and trunk. It usually begins as tender papulopustules that break down to form expanding ulcers with raised, violaceous, and well demarcated edges.1 Histopathology of pyoderma gangrenosum is non-specific but can help to differentiate it from lesions with similar features, most commonly infective, neoplastic, and rheumatological associated ulcers.4 8
Systemic steroid therapy remains the treatment of choice and leads to rapid relief of pain and initiation of healing in most patients. Infection must be excluded before starting immunosuppressive treatment.9 Successful treatment of the underlying disease results in complete remission or dramatic improvement of pyoderma gangrenosum.
Our case shows the importance of careful history taking and critical interpretation of individual findings for an accurate diagnosis and thus appropriate management. Although a retrospective diagnosis of pyoderma gangrenosum seems obvious, the report of quiescent ulcerative colitis and a traumatic cause of the ulcer was misleading. Had the non-healing traumatic ulcer been associated with a predisposing systemic disease, the diagnosis of pyoderma gangrenosum may have been reached sooner.
In fact, surgical debridement of ulcers should not be performed without confirmation of the underlying cause, as in patients with pyoderma gangrenosum it may cause extension of the necrotic area and even precipitate pyoderma gangrenosum at the donor site.7
This case emphasises the importance of connecting findings from history, examination, and investigations. The finding of anaemia should have led to the suspicion of active ulcerative colitis or at least prompted investigation as to its cause. The case also highlights that a patient's account of the history cannot always be taken at face value and, on occasion, further probing may be necessary. Finally, the case stresses that pyoderma gangrenosum should be considered in nonhealing rapidly expanding ulcers, particularly in patients with a background of related systemic disease, even with a traumatic cause.
Appropriate evaluation and critical interpretation of findings are essential for early diagnoses of pyoderma gangrenosum
Contributors: KIP and RGM searched the literature. KIP, RGM, and MGK-L wrote the article. AY managed the patient and revised the final version of the manuscript. KIP is guarantor.
Funding: None.
Competing interests: None declared.
References
Brunsting LA, Goeckerman WE, O'Leary PA. Pyoderma (ecthyma) gangrenosum. Arch Dermatol 1930;22: 655.
Schwaegerle SM, Bergfeld WF, Senitzer D, Tidrick RT. Pyoderma gangrenosum: a review. J Am Acad Dermatol 1988;18: 559-68.
Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol 1996;34: 395-409.
Wolff K, Stingl G. Pyoderma gangrenosum. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz S, eds. Fitzpatrick's Dermatology in General Medicine. New York: McGraw-Hill, 1999: 1140-8.
Bernstein CN, Blanchard JF, Rawsthorne P, Yu N. The prevalence of extraintestinal diseases in inflammatory bowel disease: a populationbased study. Am J Gastroenterol 2001;96: 1116-22.
Langholz E. Ulcerative colitis: an epidemiological study based on a regional inception cohort, with special reference to disease course and prognosis. Dan Med Bul 1999;46: 400-15.
Long CC, Jessop J, Young M, Holt PJA. Minimizing the risk of post-operative pyoderma gangrenosum. Br J Dermatol 1992;127: 45-8.
Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985;55: 173-86.
Chow RKP, Ho VC. Treatment of pyoderma gangrenosum. J Am Acad Dermatol 1996;34; 1047-60.(K I Papageorgiou, senior house officer1,)
Correspondence to: K I Papageorgiou papageorgiouk@doctors.org.uk
Pyoderma gangrenosum is a poorly understood destructive cutaneous disorder, characterised by progressive painful ulceration.1 Accurate epidemiological data are missing, but in half of cases there is an associated underlying disease, most commonly inflammatory bowel disease, rheumatological and haematological disorders.2 3 As pyoderma gangrenosum is not commonly encountered by clinicians, the diagnosis of such lesions is not always straightforward. We report the case of a man with ulcerative colitis, who presented with a non-healing ulcer of traumatic origin unresponsive to conservative and surgical management. This case emphasises the importance of detailed history taking and the consideration of pyoderma gangrenosum as a differential of such lesions in patients with a background of related systemic disease.
Case report
A 49 year old man was referred to the department of plastic surgery with a two week history of a post-traumatic, painful, and expanding ulcerated area on the anteromedial aspect of the left lower limb. The patient had a past medical history of ulcerative colitis, which he reported to be in remission for several years and he was currently not on any medication, including steroids. On initial presentation, the lesion was well demarcated measuring 6 x 4 cm with slightly undermined edges. Examination was otherwise unremarkable and conservative management was initiated. A week later, the lesion expanded to 12 x 8 cm, after which we debrided the wound and covered it with a split thickness skin graft. The postoperative course was uneventful, and the patient was discharged.
Six months later, the patient returned with a new ulcerated lesion, adjacent to the previous skin graft. Again, it was reported as post-traumatic. The lesion measured 5 x 8 cm (figure) and was indurated with violaceous undermined edges, atypical of a traumatic ulcer. Cultures were sterile and blood tests showed a normocytic anaemia and raised erythrocyte sedimentation rate.
Ulcerated lesion measuring 5 x 8 cm, on the middle third of the anterolateral aspect of the lower left leg, showing well demarcated violaceous edges
Cytological examination of scrapings from the ulcer base showed inflammatory features, and there was no bony involvement on radiography. The atypical features of the lesion and the abnormality of the blood tests prompted us to revisit the history. In depth questioning found that the ulcerative colitis was not inactive as reported and the patient was in fact experiencing bleeding from the rectum and had neglected to take his recently prescribed mesalazine and prednisolone.
Based on these findings, we considered the possibility of pyoderma gangrenosum as a differential, and we took an incisional biopsy of the lesion. Histopathology showed oedema, lymphocytic vasculitis, and neutrophilic infiltrates—features consistent with pyoderma gangrenosum.
The patient promptly started a course of 40 mg of oral prednisolone once daily and topical clobetasol. After a month, the ulcer had almost completely healed. In retrospect, the initial rapidly expanding ulcer that was treated with a skin graft was probably undiagnosed pyoderma gangrenosum.
Discussion
The diagnosis of pyoderma gangrenosum is based primarily on clinical presentation and course; histology can also be of value. The causes of pyoderma gangrenosum are unknown, but autoimmune mechanisms are probably implicated.4 Inflammatory bowel disease is the most common underlying disorder and is found in 10-15% of pyoderma gangrenosum cases.5 Of those with active ulcerative colitis, 7.1% may develop ulcerative colitis as an extraintestinal manifestation.6 Occurrence at sites of trauma or surgery is rare but well documented.7
Pyoderma gangrenosum most commonly occurs in adults aged 30-50, on the lower extremities and trunk. It usually begins as tender papulopustules that break down to form expanding ulcers with raised, violaceous, and well demarcated edges.1 Histopathology of pyoderma gangrenosum is non-specific but can help to differentiate it from lesions with similar features, most commonly infective, neoplastic, and rheumatological associated ulcers.4 8
Systemic steroid therapy remains the treatment of choice and leads to rapid relief of pain and initiation of healing in most patients. Infection must be excluded before starting immunosuppressive treatment.9 Successful treatment of the underlying disease results in complete remission or dramatic improvement of pyoderma gangrenosum.
Our case shows the importance of careful history taking and critical interpretation of individual findings for an accurate diagnosis and thus appropriate management. Although a retrospective diagnosis of pyoderma gangrenosum seems obvious, the report of quiescent ulcerative colitis and a traumatic cause of the ulcer was misleading. Had the non-healing traumatic ulcer been associated with a predisposing systemic disease, the diagnosis of pyoderma gangrenosum may have been reached sooner.
In fact, surgical debridement of ulcers should not be performed without confirmation of the underlying cause, as in patients with pyoderma gangrenosum it may cause extension of the necrotic area and even precipitate pyoderma gangrenosum at the donor site.7
This case emphasises the importance of connecting findings from history, examination, and investigations. The finding of anaemia should have led to the suspicion of active ulcerative colitis or at least prompted investigation as to its cause. The case also highlights that a patient's account of the history cannot always be taken at face value and, on occasion, further probing may be necessary. Finally, the case stresses that pyoderma gangrenosum should be considered in nonhealing rapidly expanding ulcers, particularly in patients with a background of related systemic disease, even with a traumatic cause.
Appropriate evaluation and critical interpretation of findings are essential for early diagnoses of pyoderma gangrenosum
Contributors: KIP and RGM searched the literature. KIP, RGM, and MGK-L wrote the article. AY managed the patient and revised the final version of the manuscript. KIP is guarantor.
Funding: None.
Competing interests: None declared.
References
Brunsting LA, Goeckerman WE, O'Leary PA. Pyoderma (ecthyma) gangrenosum. Arch Dermatol 1930;22: 655.
Schwaegerle SM, Bergfeld WF, Senitzer D, Tidrick RT. Pyoderma gangrenosum: a review. J Am Acad Dermatol 1988;18: 559-68.
Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol 1996;34: 395-409.
Wolff K, Stingl G. Pyoderma gangrenosum. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz S, eds. Fitzpatrick's Dermatology in General Medicine. New York: McGraw-Hill, 1999: 1140-8.
Bernstein CN, Blanchard JF, Rawsthorne P, Yu N. The prevalence of extraintestinal diseases in inflammatory bowel disease: a populationbased study. Am J Gastroenterol 2001;96: 1116-22.
Langholz E. Ulcerative colitis: an epidemiological study based on a regional inception cohort, with special reference to disease course and prognosis. Dan Med Bul 1999;46: 400-15.
Long CC, Jessop J, Young M, Holt PJA. Minimizing the risk of post-operative pyoderma gangrenosum. Br J Dermatol 1992;127: 45-8.
Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985;55: 173-86.
Chow RKP, Ho VC. Treatment of pyoderma gangrenosum. J Am Acad Dermatol 1996;34; 1047-60.(K I Papageorgiou, senior house officer1,)