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Symmetrical, painful ulceration of the lower limbs in a vascular surgery ward: a diagnostic challenge. Pyoderma gangrenosum associated with IgG-kappa paraproteinaemia.

We describe a 61-year-old patient who had been suffering from chronic ulcers of both legs for 18 months. Initially, his condition was diagnosed as ischaemic because of an ankle-brachial index of 0.6, as confirmed by additional angiography. A successful femoro-infragenual bypass procedure was performed, but the ulcers increased in size and number. He was then extensively analysed for a possible (macro)vascular origin of his symptoms. Angiographic analysis of both legs showed no arterial stenosis or occlusion. Despite the extensive experience of the vascular surgeons with leg ulcers, consultations by internal medicine, vascular medicine and dermatology, and tissue examination by our pathologists, pyoderma gangrenosum was not recognised. During a multidisciplinary meeting one of the specialists, to whom the lesions were shown, immediately considered the diagnosis on clinical grounds. The additional finding of IgG-kappa paraproteinaemia and improvement of the ulcers on treatment with corticosteroids were consistent with the diagnosis. Although the majority of patients on the vascular surgery ward have ulcers caused by ischaemia or a combined arterial/venous origin, another (rare) cause, namely pyoderma gangrenosum in association with IgG-kappa paraproteinaemia without the presence of multiple myeloma, should be taken into account.

Chronic Disease↗

Pyoderma gangrenosum as a cutaneous manifestation of diverticular disease.

We have described a case of pyoderma gangrenosum due to diverticular disease. The patient was an elderly woman with a 3-month history of cutaneous disease without associated gastrointestinal complaints. There was no history of inflammatory bowel disease, malignancy, or seropositive arthritis. Evaluation revealed a retroperitoneal abscess due to a ruptured diverticulum. Surgical resection of the affected colon led to rapid and complete resolution of all symptoms. Pathologic examination of resected tissue showed diverticulitis without evidence of chronic inflammatory bowel disease.

Aged↗

Postsurgical, chronic, nonprogressive, cutaneous ulcers: a possible variant of pyoderma gangrenosum associated with diabetes mellitus.

Two patients developed persistent ulcers on the trunk after cutaneous surgery. Both had "chemical" diabetes mellitus. Bacteriologic and histopathologic studies of the ulcers were not revealing of cause. The characteristics of the ulcers are described, and are contrasted with typical lesions of pyoderma gangrenosum and Meleney's postoperative progressive synergistic bacterial gangrene. We believe these patients had variant lesions of pyoderma gangrenosum.

Adolescent↗

[Pyoderma gangrenosum and breast cancer: a new case].

We report here a new case of pyoderma gangrenosum (PG) associated with a breast cancer in a 39-year-old woman. We only found in literature three other reports of this rare entity which seems usually to be associated with monoclonal gammopathy, gastro-intestinal diseases such as Crohn's disease, chronic ulcerative colitis, leukemias or rheumatologic diseases. A commun hapten between of tumor and skin may explain the origin of this inflammatory lesion. In our case, PG could be a paraneoplastic syndrome.

Adult↗

[Peristomal pyoderma gangrenosum in a patient with ulcero-hemorrhagic rectocolitis].

Cutaneous manifestations of chronic ulcerative colitis are numerous and present in 20 p. 100 of the patients. The case of a 49-year old man with peristomal pyoderma gangrenosum (PG) is presented. In November, 1983, one year after the onset of chronic ulcerative colitis, the patient underwent total colectomy. The formation of an ileal reservoir resulted in a pelvic abscess which necessitated right lower ileostomy. Because of severe ulcerations around the stoma, the ileostomy was transferred to the left lower abdomen. Similar inflammatory ulcerations developed, and the diagnosis of pyoderma gangrenosum was made. The patient responded very well to dapsone combined with prednisone for only 6 months. The possibility of PG being an autoimmune condition is discussed, as well as the differential diagnosis. The several therapeutic approaches are summarized. The peristomal localization of PG in this patient suffering from chronic ulcerative colitis seems to be rather unique. No other case has been reported so far, to our knowledge.

Administration, Topical↗

[Pyoderma gangrenosum: and important differential diagnosis from wound infection. Case report of a life threatening course].

Pyoderma gangrenosum (PG) is a necrotizing and ulcerative skin disease of unknown cause. The pathogenesis is thought to be related to a defective immune response. The ulcerations appear spontaneously or after skin trauma. Development in a surgical wound can be misinterpreted as wound infection. However, in contrast to postoperative infections, treatment of PG requires immunosuppressive therapy. We report a case of PG following hip-joint surgery that was complicated by a severe life-threatening systemic inflammatory response syndrome (SIRS). Typically, wound and blood cultures were negative for pathogens. Prednisolone stopped the activity of the disease promptly.

Anti-Inflammatory Agents↗

Effective steroid-sparing treatment for peripheral ulcerative keratitis and pyoderma gangrenosum.

OBJECTIVE: To report a patient with a rare clinical presentation of marginal keratitis and pyoderma gangrenosum (PG) that responded to the steroid-sparing agent azathioprine. DESIGN: Interventional case report. METHODS: Case report and MEDLINE review of the medical literature on PG-associated peripheral ulcerative keratitis (PUK) and its treatment. RESULTS: A patient with a biopsy-proven history of PG developed an explosive episode of PUK. The ocular inflammation was minimally responsive to topical therapy and required significant long-term oral steroids for control. The patient's active skin lesions and marginal keratitis responded dramatically to systemic oral azathioprine. Since the initiation of treatment with azathioprine, there have been no subsequent recurrences of PG or marginal keratitis. CONCLUSIONS: PUK may be associated with other inflammatory conditions, such as PG and arthritis. Systemic azathioprine may successfully control ocular and extraocular conditions and should be considered in the clinical setting of topical therapy failure and/or extraocular inflammatory conditions.

Administration, Oral↗

A case of pyoderma gangrenosum on the stump of an amputated right leg.

We present here a case of pyoderma gangrenosum (PG) on the stump of an amputated leg. The patient was a 69-year-old woman who had both of her legs amputated due to acute arterial occlusion. An ulcer first appeared nine years later, after which point it continued to fluctuate in size. Complications included regional blood flow disorder at the amputated stump, diabetes, and secondary infection. Despite various therapies, the ulcer exacerbated, and hypoproteinemia, increased CRP, and fever were confirmed. The patient was diagnosed as having PG based on her clinical symptoms and because the ulcer did not respond to various therapies. The ulcer improved significantly in response to administration of 40 mg/day of prednisolone, and complete epithelialization was later achieved. Given the presence of multiple complications, it was extremely difficult to confirm PG. Therefore, it is important for physicians to consider PG as one of the causes of intractable ulcers.

Aged↗

Pyoderma gangrenosum treated with high-dose intravenous immunoglobulins: Two cases and review of the literature.

Pyoderma gangrenosum (PG) is a neutrophilic skin disease commonly treated with immunosuppressants. High-dose intravenous immunoglobulins are used to treat a range of inflammatory diseases, but we found only five reports of the use of high-dose intravenous immunoglobulins in the treatment of PG. We report on two patients with PG for whom immunosuppressants could not be prescribed and who were treated with high-dose intravenous immunoglobulins. Case 1 was a 58-year-old man who presented with a 6-year history of PG. He was initially treated with prednisone. The 20 mg/day dosage of prednisone could not be reduced and treatment had to be discontinued after 1 year because of serious adverse effects. Minocycline treatment led to improvement but had to be discontinued after 6 years because of facial skin hyperpigmentation. Case 2 was a 66-year-old man who presented with a 3-year history of PG. Different therapeutic procedures for PG (prednisone, topical tacrolimus or betamethasone) had failed. High-dose intravenous immunoglobulins were administered monthly at a dose of 2 g/kg for 6 months. The treatment induced stabilisation of the disease and made it possible to reduce corticosteroid use in both patients. These cases show that high-dose intravenous immunoglobulins represent a therapeutic alternative for PG, but the efficacy of this treatment should be confirmed in further studies.

Aged↗

Successful treatment of pyoderma gangrenosum that developed in a patient with myelodysplastic syndrome.

We describe the successful treatment of pyoderma gangrenosum (PG) that developed in a patient with myelodysplastic syndrome (MDS). A 63-year-old Japanese man with MDS was admitted to our hospital because of a large skin ulcer on his neck in November 2001. The initial diagnosis was infectious dermatitis, and antimicrobial therapy was performed, using imipenem/cilastatin, isepamicin, and amphotericin B. However, this therapy was not effective, and the lesion worsened. Cultures of blood, throat swab, and ulcer pus yielded no microorganisms. A biopsy of the skin lesion revealed a severe infiltration of neutrophils in the dermis, without any evidence of infection. The lesion was finally diagnosed as PG, and systemic administration of corticosteroid hormone was started in December 2001. The patient was initially pulsed with 1 g methylprednisolone daily for 3 days. The dose was immediately reduced, and the treatment was maintained with 30 mg prednisolone daily. The skin lesion responded markedly to the therapy, and C-reactive protein became negative. The patient was discharged in February 2002 because the lesion was almost cured. Prednisolone administration was tapered after 6-month maintenance therapy. No recurrence of PG was seen, although his MDS transformed into leukemia in April 2003. Only 31 cases of MDS developing PG have been reported in the past 20 years in Japan. This report describes one such rare patient who was successfully treated with the use of high-dose pulse methylprednisolone and long-term maintenance therapy.

Diagnosis, Differential↗

Pyoderma gangrenosum following cytosine arabinoside, aclarubicin and granulocyte colony-stimulating factor combination therapy in myelodysplastic syndrome.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis, which may be associated with systemic conditions such as hematologic disorders. We present a patient who had been diagnosed as having myelodysplastic syndrome associated with PG at onset, in whom a febrile ulcerative skin lesion developed following cytosine arabinoside, aclarubicin and granulocyte colony-stimulating factor (G-CSF) combination chemotherapy in the course of the disease. Skin biopsy revealed dense neutrophilic infiltrate in the dermis with central epidermal ulceration, consistent with the diagnosis of PG. Oral prednisolone was effective for the skin lesion. In this case, G-CSF application may participate in the recurrence of PG.

Aclarubicin↗

Pyoderma gangrenosum associated with selective hereditary IgA deficiency.

A case of pyoderma gangrenosum is described in a girl aged 4. The condition was associated with selective IgA deficiency. The father and the 2 brothers suffered from the same deficiency (autosomal dominant transmission). Treatment with prednisolone and clofazimine produced an excellent clinical response.

Child, Preschool↗

Pyoderma gangrenosum associated with nasal septal perforation, oropharyngeal ulcers and IgA paraproteinemia.

We report a case of pyoderma gangrenosum (PG) associated with nasal septal perforation, pharyngeal ulcers and IgA paraproteinemia. A 28-year-old woman first developed painful undermined ulcers on her perianal, inguinal and axillary areas when she was 22 years old. Histological findings from the cutaneous ulcers showed dermal and epidermal infiltrate of neutrophils, which was compatible with PG. Laboratory examinations did not detect any associations of systemic diseases other than polyclonal IgA paraproteinemia. Nasal fiberscopy revealed septal perforation and multiple ulcers on her pharynx. The biopsy specimen from the pharyngeal ulcers showed a polymorphous cellular infiltrate without necrotizing vasculitis or granuloma. However, there were no atypical lymphocytes that are typically seen in nasal NK/T lymphoma. By immunohistochemical analysis, the infiltrated lymphocytes were proved to be T cells and Epstein-Barr virus encoded RNA (EBER) was not detected. No pulmonary or renal lesions resembling Wegener's granulomatosis were found. Taken together, the nasal septal perforation was considered as nasal involvement of PG.

Adult↗