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We describe a patient with long-standing pyoderma gangrenosum unresponsive to therapy. The patient had concomitant cryoglobulinemia and hepatitis C. When the hepatitis C was treated with interferon alfa-2a his pyoderma gangrenosum resolved. Whether this was from the interferon alfa or spontaneous resolution is not known.
Critical to the proper management of pyoderma gangrenosum are correct diagnosis, identification and treatment of any underlying disorder, and the proper choice of topical and systemic therapy. Many agents are available for the treatment of pyoderma gangrenosum. We review the current therapeutic options, their efficacy and side effects, and we offer some guidelines for their proper selection.
This report describes a patient with extensive pyoderma gangrenosum in whom there were co-existent lung abnormalities. The patient's X-ray showed peripherally sited multiple pulmonary lesions bilaterally. A lung biopsy showed chronic non-specific inflammatory changes with neutrophil and lymphocyte infiltration which were similar to the skin lesions. This case was diagnosed as multiple aseptic nodules in pyoderma gangrenosum. The pulmonary infiltrative shadows were controlled only with prednisolone treatment. Steroid therapy is considered to be the first choice to control pulmonary lesions of this disease.
A 60-year-old male patient developed progressive wound ulcerations, simulating wound sepsis after coronary bypass operation. The condition did not respond to intensive antibiotic therapy. Based on clinical signs and biopsy, the diagnosis of pyoderma gangrenosum was made and successfully treated with cyclosporin A. Pyoderma gangrenosum, although extremely rare, must be considered as a possible diagnosis in wound complications unresponsive to traditional therapy.
BACKGROUND: X-linked agammaglobulinemia (XLA), characterized by a profound deficiency of all immunoglobulins and the absence of mature B cells, is caused by mutations in the gene encoding Bruton tyrosine kinase (BTK). Most patients have recurrent sinopulmonary infection. Infections usually occur in multiple locations across time, but single infection may be limited to one anatomic location. OBJECTIVES: To report a case of atypical XLA with recurrent pyoderma and to observe the immunologic changes in the patient in 10 years. METHODS: Immunologic investigations, skin wound culture, and molecular study with DNA sequencing were performed. RESULTS: The patient was originally diagnosed as having common variable immunodeficiency disease because of the presence of circulating B cells (CD19+ B cells: 7%) at 11 years old. On further evaluation at the age of 20 years, flow cytometric analysis of lymphocytes showed only 0.4% B cells. The molecular study with DNA sequencing of the patient showed a point mutation in complementary DNA 1630 A>G(p.R544G) in the BTK gene, indicating that the patient has XLA. The mutation analysis of the BTK gene revealed a normal DNA sequence in the other family members. CONCLUSIONS: This case is an important example of a possible presentation of XLA with a predominant skin manifestation, and it demonstrates that maintaining a high level of clinical suspicion is essential for the diagnosis of XLA in a child with recurrent pyoderma.
Dermatology and internal medicine have in common that many systemic diseases manifest with skin symptoms that are easily accessible for both diagnostic and investigative procedures. Even if modern practice of medicine tends towards organ specific particularity of specialists, dermatology traditionally strives for interdisciplinary communication. Cutaneous manifestations of internal diseases present in various forms. In a syndrome, a number of symptoms present in an identical pattern, and pattern recognition is diagnostic. Vice versa, a distinct skin sign can be a clue to various internal disorders, that have to be considered in the differential diagnosis. Purpura and livedo due to either inflammatory (immune complex mediated or associated with ANCA) or vasoocclusive microthrombotic vascular disease are presented as examples. Finally, subtile variations in key symptoms of the skin, such as of pyoderma gangrenosum, may give a clue to a specific underlying disease, such as rheumatoid arthritis, inflammatory bowel disease, monoclonal gammopathy, or hematologic disease, as exemplified by the variants of pyoderma gangrenosum.
A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.
We report two patients with pyoderma gangrenosum complicated with nasal septal perforation. An 18-year-old woman and a 65-year-old man had typical lesions of pyoderma gangrenosum on the legs that responded well to oral prednisolone. Both patients complained of mild nasal discharge, and nasal fibroscopy revealed nasal septal perforation. Biopsy of the nasal lesions showed an active inflammatory infiltrate, mainly of neutrophils. Systemic investigations failed to show any pulmonary or renal lesions of Wegener's granulomatosis. Cytoplasmic immunofluorescent pattern antineutrophil cytoplasmic antibody was negative. In both cases, intense neutrophilic infiltration was observed not only in skin lesions but also in nasal lesions, which may indicate that the nasal lesions had a pathogenesis in common with the skin lesions.
We report a patient with combined cutaneous and oropharyngeal pyoderma gangrenosum in association with an IgA lambda paraproteinaemia. The differential diagnosis of oral pyoderma gangrenosum is discussed.
Peristomal pyoderma gangrenosum (PPG) is a variant of pyoderma gangrenosum (PG) that is more refractory to treatment. It is a cause of severe morbidity and poses a therapeutic challenge for the clinician. Infliximab (Remicade(R); Centocor, Malvern, PA, USA) is a chimeric monoclonal antibody directed against tumour necrosis factor-alpha that has been proven to be effective in the treatment of inflammatory bowel disease (IBD) and rheumatoid arthritis. Currently, very few reports exist documenting its use in the treatment of PG and none in the treatment of PPG. We describe our experience of treating three patients with IBD-associated PPG with infliximab. All patients tolerated the drug without significant side-effects. Two patients with PPG recovered completely following the administration of infliximab, and one patient had a partial response to the drug. We conclude that infliximab appears to be a safe and effective therapeutic alternative in patients with PPG.
We present a patient with both pyoderma gangrenosum in a thoracotomy wound and a pulmonary cavitating lesion with the histological features of Wegener's granulomatosis. An ulcer with blue undermined edges developed in our patient's thoracotomy scar after a lobectomy was carried out for the cavitating lung lesion and c Antineutrophil Cytoplasmic Antibodies (cANCA), which is highly specific for active Wegener's granulomatosis but was negative at the time of her skin lesion. This suggests that our patient had cutaneous pyoderma gangrenosum rather than cutaneous Wegener's granulomatosis, although the two conditions may occasionally have similar clinical and histological features.
A 74-year-old woman was referred to our department in December 1999 for a pyoderma gangrenosum (PG) arising at the edges of chronic leg ulcer. The history was positive for benign monoclonal gammopathy, ischemic hypertensive cardiopathy, polyarthrosis and venous lower leg deficiency. Monoclonal gammopathy of IgA Kappa type was diagnosed 10 years before with a continued benign nature. In 1990 a post traumatic PG of the left leg was diagnosed and a therapy with Cyclosporine A was started with healing of the lesion. In June 1999, 6 months before the hospitalization, a typical venous ulcer of the right leg appeared and was treated with bed-rest, compression bandaging and topical desloughing therapy. In the last month, after a minor surgical debridement of the wound, the lesion developed pustules evolving into a painful, necrotic ulcer with ragged, purple-red, undetermined borders (Fig. 1). A relapse of PG was suspected. Histological examination was consistent with pyoderma gangrenosum and showed massive neutrophilic infiltration, hemorrhage and necrosis of the overlying epidermis. Wound culture was negative. Other laboratory examinations only showed IgA = 8.15 g/L. Investigation of other underlying medical conditions were normal or negative. The venous leg ulcer gradually healed with antiseptic and compression-bandage therapy. After a 4-month course of topical steroid therapy with good results, the PG recurred also involving the proximal area of the leg. Methylprednisolone 50 mg/day was started. Healing began 10 days later and 2 months later the wound healed and epithelialized. Steroid was reduce to 5 mg daily for 4 months. No recurrence was seen when the drug was stopped.
Pyoderma gangrenosum sometimes takes a recalcitrant course that is unresponsive to standard immuno-suppression with corticosteroids and/or cyclosporin A. In these cases improvement of painful ulcerations is a therapeutic challenge. We report a 17-year-old boy with severe pyoderma gangrenosum treated successfully with mycophenolate mofetil and autologous keratinocyte transplantation using an esterified hyluronic acid delivery system.
We present the case of a 59-year-old woman who had large ulcerations on her right leg that were diagnosed initially as pyoderma gangrenosum and treated with three immunosuppressive agents (cyclosporin, prednisone and azathioprine) for 6 months. Results of a biopsy at 6 months showed numerous cigar-shaped bodies consistent with Sporothrix schenckii; identification was confirmed by tissue culture. A retrospective review was performed for all cases diagnosed as sporotrichosis from tissue culture or biopsy specimens at the Mayo Clinic. Nineteen cases were identified. The present case was the only one in which fungal organisms were visible on histological examination. The present case emphasizes the importance of making a definitive histological diagnosis in unusual ulcer cases or in suspected cases of pyoderma gangrenosum before the initiation of immunosuppressive therapy. The large number of cigar-shaped bodies in the tissue is a rare finding in sporothrix infection and has been reported in only two cases previously.
Postoperative pyoderma gangrenosum (PPG), also known as postoperative progressive gangrene of Cullen, is a rare and rapidly evolving complication of surgical procedures. Since the first description by Cullen in 1924 (Surg Gynecol Obstet 1924; 38: 579-582) various case reports have been published. Even in typical cases PPG is often misdiagnosed and therefore wrongly treated; the unknown aetiology makes treatment difficult. The therapies used for pyoderma gangrenosum include systemic corticosteroids, azathioprine, dapsone, mercaptopurine, sulphasalazine, sulphapyridine, thalidomide, cyclophosphamide, clofazimine, isotretinoin, immunoglobulins and cyclosporin. We report on two patients with PPG following breast surgery who were successfully treated with low-dose cyclosporin (2.5-5 mg/kg per day).
We report a case of atypical bullous pyoderma gangrenosum associated with acute myeloid leukaemia in which we found atypical myeloid cells within the skin lesion. Although there have been many reported cases of leukaemia-associated pyoderma gangrenosum, the finding of myeloblasts in the skin has rarely been described.
Having previously associated metabolic oscillations with cell locomotion, we hypothesized that patients with abnormalities in neutrophil trafficking may display aberrant intracellular oscillations. A pyoderma gangrenosum patient exhibiting aberrant leukocyte trafficking in vivo and skin ulceration without infection was identified. This patient's neutrophils constitutively overexpressed and clustered the leukocyte integrins CR3 and CR4 and failed to display appropriate integrin-to-GPI receptor interactions. Increased levels of tyrosine phosphorylation were observed. NAD(P)H oscillations, which are sinusoidal in normals, were chaotic with multiple frequency components in this patient's neutrophils. Normal cell shape and sinusoidal NAD(P)H oscillations were restored by providing a pulsed electric field to drive metabolic oscillations and by temperature reduction. N-acetyl-D-glucosamine disrupted CR3 clusters and sinusoidal NAD(P)H oscillations returned. Anecdotal reports suggest that local hypothermia is clinically useful for this patient. These data define the first metabolic oscillation-associated disease and suggest that pyoderma gangrenosum can be classified as a dynamical disease at the cellular level.
BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon necrotising, non-infective ulceration of the skin. The management of PG is aimed at limiting tissue destruction, promoting the healing of the wound, and providing an acceptable cosmetic result. However, skin grafting is normally avoided because of the potential risk of pathergy-the localization of skin damaged by trauma. REPORT: We describe the use of split skin grafts in the management of ulcerative pyoderma gangrenosum in 4 patients. RESULTS: Our cases demonstrate that split skin grafts are a useful treatment modality in patients with ulcerative PG, producing a good cosmetic result. One case illustrates the importance of ensuring the disease is quiescent prior to grafting, to avoid pathergy. The other cases emphasise the need for prolonged immunosuppressive therapy to minimise the chance of reactivation of the disease process. CONCLUSION: Our preliminary experience of 4 cases of ulcerative PG indicates that split skin grafts have a role to play in its management. The ultimate cosmetic result is considered to be superior to allowing the wound to heal by secondary intention. To limit the risk of pathergy developing, our experience suggests a role for prolonged courses of immunosuppressive therapy. The most effective dose and duration of immunosuppressive therapy in patients with PG treated with split skin grafts remains to be determined. A controlled study would be of benefit to compare it with other current treatment options.