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SAPHO syndrome and pyoderma gangrenosum: is it fortuitous?

Pyoderma gangrenosum is well known as an associated feature of inflammatory bowel disease (IBD). Recently, higher than normal prevalence of IBD in patients with the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome has been reported. However, the association of pyoderma gangrenosum with SAPHO syndrome without definitely excluded IBD has not been reported. We describe a case that suggests a possible connection between these 2 entities.

Acne Vulgaris↗

Cyclosporine for pyoderma gangrenosum.

A case of pyoderma gangrenosum that occurred in the absence of underlying illness and was initially misdiagnosed is described. Hemorrhagic pustules and ulcers appeared over vein-graft sites after open heart surgery and were treated with cyclosporine. The result was rapid and complete resolution. It is the purpose of this report to emphasize pyoderma gangrenosum as a cause of ulceration in healthy individuals and to highlight the addition of cyclosporine to the therapeutic armamentarium.

Cyclosporine↗

Successful treatment of postoperative pyoderma gangrenosum with cyclosporin.

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).

Adult↗

Pyoderma gangrenosum producing saddle nose deformity.

Pyoderma gangrenosum affecting the nose is rare and this may lead to diagnostic confusion because of the large differential diagnosis. As diagnosis is made, largely, on the basis of exclusion the treatment of pyoderma gangrenosum may be unduly delayed. The condition is often disfiguring, particularly following inappropriate surgical intervention, and early diagnosis is therefore important. We present a case of pyoderma gangrenosum managed initially in the community with minor surgery and resulting in the rare complication of saddle nose deformity.

Aged↗

Familial ulcerative pyoderma gangrenosum: a report of 2 kindred.

Pyoderma gangrenosum is a rare, chronic ulcerative skin disease. It is a diagnosis of exclusion, after ruling out other causes of cutaneous ulceration. The etiology of pyoderma gangrenosum is poorly understood but is likely multifactorial. We describe 2 families affected by ulcerative pyoderma gangrenosum. This familial clustering suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.

Adult↗

Pyoderma gangrenosum following cesarean delivery.

BACKGROUND: Pyoderma gangrenosum is a rare, chronic inflammatory disease that can develop after minor trauma or surgery. Most reports of this condition developing after abdominal surgery have been reported following ostomy formation. A number of systemic diseases have been associated with this disorder, most commonly inflammatory bowel disease, though 40-50% of cases have no associated systemic disease. CASE: A 32-year-old woman developed spiking fever and purulent appearing wound discharge with a rolled erythematous border after cesarean delivery. Her condition worsened despite various regimens of intravenous antibiotics and local treatment. Pyoderma gangrenosum was diagnosed. Treatment with steroids resolved the lesion, and associated systemic diseases were not found. CONCLUSION: Consideration of the diagnosis of pyoderma gangrenosum in cases of apparent wound infections unresponsive to antibiotics should result in early diagnosis and treatment of this condition.

Adult↗

Optimal treatment of pyoderma gangrenosum.

The optimal treatment of pyoderma gangrenosum includes a combination of local wound care and systemic medications. Oral and pulse intravenous corticosteroids have traditionally been the most commonly recommended first-line systemic therapies. Cyclosporine, with or without corticosteroids, has more recently emerged as a first-line systemic treatment. A multitude of immunosuppressive and immune-modulating medications, as well as antimicrobial agents with anti-inflammatory properties have also been widely prescribed. Often, it is difficult to achieve control of aggressive cases of pyoderma gangrenosum, necessitating administration of a combination of systemic therapies. Furthermore, patients recalcitrant to one or many medications are frequently reported. Concomitant disease, intolerance to a class of medications, and the patient's response to prior therapies can help guide a practitioner in choosing the optimal treatment of pyoderma gangrenosum.

Adjuvants, Immunologic↗

Pyoderma gangrenosum associated with primary thrombocythaemia.

Pyoderma gangrenosum is most commonly associated with inflammatory bowel disease and rheumatoid arthritis, but it has been associated with various haematological malignancies. A 54-year-old man with no history of bowel disease or arthritis presented with a leg ulcer, which healed after treatment. Results of bone marrow aspiration were compatible with primary thrombocythaemia. Seven weeks later there were pronounced recurrence of the ulceration and pyoderma gangrenosum was diagnosed. The appearance of pyoderma gangrenosum associated with blood disorders may differ from that associated with bowel and joint disease.

Humans↗

Pyoderma gangrenosum in a patient with essential thrombocythemia.

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. OBJECTIVE: This article is a review of the associations between pyoderma gangrenosum and other diseases. RESULTS: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. CONCLUSION: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.

Anti-Inflammatory Agents↗

Recurrent pyoderma gangrenosum and agnogenic myeloid metaplasia.

Pyoderma gangrenosum has been associated with myelogenous leukemia and plasma cell dyscrasia. When associated with leukemia, pyoderma gangrenosum often has a distinctive clinical presentation with an advancing bullous margin. The pathogenesis of this disorder is unknown, although defective immune mechanisms may be operative. The occurrence of pyoderma gangrenosum and agnogenic myeloid metaplasia in the same patient has now been reported sufficiently to make it a recognized association.

Female↗

[Pyoderma gangrenosum: report of 11 cases].

BACKGROUND: Pyoderma gangrenosum is an ulcerative disease of the skin of unknown cause and generally associated to systemic illnesses. It requires an aggressive systemic therapy and there is little information about its long term evolution. AIM: To report the clinical features of patients with pyoderma gangrenosum. PATIENTS AND METHODS: Eleven patients (10 female) aged 27 to 81 years old with pyoderma gangrenosum are reported. All had a pathological study. Patients were followed up for a mean of 60 months. RESULTS: The lesions were located in the lower limbs in 8 patients. Pathological study showed neutrophyl infiltrates in nine patients. Three patients had inflammatory bowel disease [corrected]. During the follow up, the disease recurred in multiple occasions, in six patients, after treatment with steroids or sulphones, and one patient has had permanently active lesions. CONCLUSIONS: Pyoderma gangrenosum appears mostly in the lower limbs and has a high recurrence rate.

Adrenal Cortex Hormones↗

Atypical pyoderma gangrenosum with leukemia.

Pyoderma gangrenosum (PG) has been increasingly reported in association with myeloproliferative disorders. Monoclonal gammaopathy, myeloma, myeloid metaplasia, and polycythemia have all been found in association with PG. Recently, seven cases of PG in association with leukemia have been described: three cases with acute myeloblastic leukemia, two cases with chronic myelogenous leukemia, one case with acute lymphoblastic leukemia, and one case with acute leukemia of either plasma cell or myeloblast origin. To these we add two cases of PG with acute myeloblastic leukemia. These patients often have an atypical clinical presentation for PG, with bullae and relatively superficial involvement obscuring the correct diagnosis.

Acute Disease↗

Clofazimine. A new agent for the treatment of pyoderma gangrenosum.

Eight patients had pyoderma gangrenosum. They were given a phendimetrazine tartrate derivative, clofazimine (Lamprene [Britain]), which is a chemotherapeutic agent used mainly in certain mycobacterial infections and which also has phagocytosis-enhancing properties. The effect of this drug was remarkably good, with rapid healing of the lesions commencing 3 to 14 days after treatment was started. The mechanism for the effect of clofazimine in pyoderma gangrenosum is not known.

Adult↗

Parastomal pyoderma gangrenosum in inflammatory bowel disease.

PURPOSE: Parastomal pyoderma gangrenosum is uncommon and its association with inflammatory bowel disease is unclear. This is a review of five patients with parastomal pyoderma gangrenosum. METHODS: A retrospective review of five patients with ulcerative colitis (two patients) or Crohn's disease (three patients) who have been seen in one surgical unit was conducted. RESULTS: All patients were females and each presented within nine months of abdominal surgery and stoma construction. All had active proctitis (n = 3) or perianal Crohn's disease (n = 2). Both patients with perianal Crohn's disease had a mild clinical course with healing of parastomal pyoderma gangrenosum when treated with steroids with and without low-dose cyclosporin A. They both had curettage of the perineal wound as well. In the remaining three patients with active proctitis, the parastomal lesions failed to resolve despite high-dose systemic steroids. By contrast, the parastomal pyoderma gangrenosum healed promptly in two of these patients following proctectomy for active proctitis. CONCLUSION: The variable clinical outcome of parastomal pyoderma gangrenosum may be related to the activity of the underlying inflammatory bowel disease or possibly to low-grade perineal sepsis.

Adult↗

Treatment of pyoderma gangrenosum with cyclosporine.

BACKGROUND AND DESIGN: Pyoderma gangrenosum is a chronic inflammatory ulcerative skin disease of unknown origin, often associated with various diseases including inflammatory bowel disease, inflammatory arthritis, monoclonal gammopathies, hepatitis, and myeloproliferative disorders. Treatment of associated systemic disorders may improve the ulcers, but lesions may be recalcitrant and persist for months to years. Therapy for pyoderma gangrenosum includes high-dose systemic corticosteroids, sulfa drugs such as sulfasalazine, clofazimine, and immunosuppressive agents such as mercaptopurine and azathioprine; these drugs are sometimes ineffective. RESULTS: We present a series of 11 patients with pyoderma gangrenosum, with a wide range of underlying diseases, whose ulcers were refractory to usual therapy and who were treated with low-dose cyclosporine. Ten of the 11 patients cleared rapidly and completely with cyclosporine therapy. CONCLUSIONS: Cyclosporine should be seriously considered as a primary form of treatment for pyoderma gangrenosum.

Adult↗

Surgical management of pyoderma gangrenosum: case report and review.

BACKGROUND: Commonly used treatments for pyoderma gangrenosum are medical, with immunosuppressive agents employed most often. OBJECTIVE: To report a case and discuss the indications for radical surgical treatment of pyoderma gangrenosum. METHODS: Analysis of a case of Crohn's disease-associated pyoderma gangrenosum treated with immunosuppression followed by amputation, and a review of the literature on surgical management of pyoderma gangrenosum. RESULTS: In unstable patients with intractable multiple medical problems, surgical treatment of pyoderma gangrenosum may be indicated by the existence of these life-threatening comorbidities. The recent literature suggests that surgical management of pyoderma gangrenosum may also be appropriate in other special circumstances. CONCLUSIONS: Surgical management, including amputation, may have a role in the management of pyoderma gangrenosum. Further research is needed to delineate precisely the circumstances and patient factors that are appropriate indications for such surgery.

Aged↗

Pyoderma gangrenosum and ulcerative colitis.

The relationship of pyoderma gangrenosum and ulcerative colitis remains uncertain. We investigating 14 patients with pyoderma gangrenosum by colonoscopy with multiple biopsies. Six patients had ulcerative colitis and all of these had disease affecting the whole colon. There were no correlations between exacerbations of the colitis and the onset or course of pyoderma gangrenosum. The remaining eight patients with pyoderma gangrenosum had no other disease and they were found to be significantly older than those patients with coexisting colitis (P less than 0.002).

Adult↗

Pyoderma gangrenosum associated with biphenotypic acute leukemia.

Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We present, to our knowledge, the first case of pyoderma gangrenosum associated with biphenotypic acute leukemia wherein the malignant cells show a phenotype specific for myelogenic and lymphocytic leukemia. Histopathologic examination revealed rather nonspecific features without involvement of leukemic cells in the skin lesions. Treatment with systemic steroids was followed by characteristically rapid healing of the skin lesion.

Female↗