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Pyoderma gangrenosum as initial manifestation of Graves' disease.

Pyoderma gangrenosum (PG) is an uncommon ulcerative condition. It is most commonly associated with inflammatory bowel disease, haematological malignancies and autoimmune disease. We report on two patients have developed PG during the initial course of Graves' disease (GD), prior to treatment of this condition. The lesions completely cleared with treatment by colchicine in the first case and with oral ciclosporin in the second case. The report emphasizes that a diagnostics of PG should be considered in patients with GD and skin ulcers.

Administration, Oral↗

[Atypical pyoderma gangrenosum in 92-year-old patient].

Pyoderma gangrenosum (PG) is rather rare chronic dermatosis presenting as cutaneous necrosis followed by development of expanding chronic ulcer with well-demarcated, undermined, violaceous borders. Diagnosis of PG is predominantly based on the clinical presentation and course. Histopathologic findings may be non-specific. Half of patients suffer from a somatic systemic disease. We describe 92-year-old lady presenting with large non-healing ulcer of the anterolateral calf with the diagnosis of atypical PG. In a discussion differential diagnosis of cutaneous ulcers mimicking PG is reviewed and the treatment possibilities summarised.

Aged↗

Pyoderma gangrenosum occurring near an arteriovenous dialysis shunt.

Pyoderma gangrenosum (PG) is an uncommon cutaneous disease of unknown etiology. In 50 percent of affected patients, PG is associated with systemic disease including inflammatory bowel disease, arthritis, and hematologic malignancies.(1) Diagnosis of PG is based on clinical presentation, histopathology and on the exclusion of other diseases that can produce clinically similar lesions, e.g. infection, vasculitis, malignancy, collagen vascular diseases, diabetes, and trauma. Four variants of PG have been described: ulcerative, pustular, bullous, and vegetative.(2) We report a woman with renal failure who developed PG in the absence of any obvious triggering trauma in a distinctive unilateral crop just distal to an arteriovenous dialysis shunt.

Arteriovenous Shunt, Surgical↗

Treatment of recalcitrant ulcers in pyoderma gangrenosum with mycophenolate mofetil and autologous keratinocyte transplantation on a hyaluronic acid matrix.

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.

Adolescent↗

Management strategies for pyoderma gangrenosum: case studies and review of literature.

Pyoderma gangrenosum (PG) is a systemic disease with cutaneous manifestations consisting of necrotizing ulceration. The etiology of PG is controversial, and optimal management strategies have not been established. Current management is primarily medical to control the systemic inflammatory process, with occasional surgical intervention at the ulcer site. Based on the current literature and on the authors' clinical experience, the optimal outcome depends on early diagnoses and a combination of medical and surgical therapy. Initial management is directed toward medical control of the inflammatory process and local wound care. Surgical strategies involve recipient site preparation via local wound care and serial allograft followed by autologous skin graft or muscle flap coverage when necessary. Long-term wound stabilization is obtained only through control of the systemic and local inflammatory process.

Aged↗

Pyoderma gangrenosum of the eyelid and acute rhinosinusitis.

Pyoderma gangrenosum (PG) is a destructive, necrotizing, noninfective ulceration of the skin. Periorbital PG is extremely rare, and may progress, with eventual loss of the eye. We report a 47-year-old woman who presented with a right periorbital swelling of 8 days duration and concurrent acute rhinosinusitis. A clinical diagnosis of right periorbital PG was made, and treatment instituted with intravenous methylprednisolone and antibiotics, resulting in rapid resolution of the condition. The clinical features in this case, together with the past history of histologically confirmed PG, enabled a rapid diagnosis to be made, with early administration of treatment resulting in minimal scarring. The clinical picture could easily be confused with periorbital cellulitis, and this case demonstrates the importance of considering the differential diagnoses of periorbital swelling.

Acute Disease↗

Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features.

BACKGROUND: Classical pyoderma gangrenosum (CPG) is an uncommon, idiopathic, neutrophilic skin disease, generally associated with other skin conditions and systemic diseases, and with several treatment options. OBJECTIVE: The objective of this review was to know the clinical and therapeutic features of our cases of CPG. METHODS: Twenty-six cases of CPG were included; 65 episodes of PG and 120 treatment courses were analysed; and the mean follow-up was 4.6 years. RESULTS: 21 cases (80%) had lesions on the lower extremities, 16 (61%) had recurrences, 22 (84%) had some disease associated, and 13 (50%) had autoimmune inflammatory conditions. Cyclosporin A (CSA) was used in 22 (84%) patients and 51 (78%) episodes, and prednisone (PDN) was used in 15 (57%) patients and 26 (40%) episodes. CONCLUSIONS: CSA efficacy was excellent, with an early response and acceptable toxicity, although it did not seem to have any impact on the incidence of PG recurrences.

Adolescent↗

Pyoderma gangrenosum: a report of 21 cases.

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon, destructive, cutaneous ulceration, belonging to the neutrophilic disease spectrum. It is associated with systemic disease in 50% of cases. METHODS: We report a retrospective study of 21 cases of PG. All cases studied fulfilled the following criteria: (i) clinical features of PG; (ii) histopathology consistent with a diagnosis of PG, and excluding other specific dermatoses. RESULTS: The average age of our patients was 41.8 years. The male to female ratio was 1.1. The typical ulcerative variant was found in 17 patients, bullous PG in two patients, and the granulomatous variant in two patients. Sixty-two per cent of our patients had lesions on their lower legs. Two patients had neutrophilic pulmonary involvement concurrent with the ulcers. An association with other internal diseases was noted in 12 patients. Histopathologic study showed vasculitis in 13 patients. Of these, 11 were leukocytoclastic and the others predominantly lymphocytic. CONCLUSIONS: PG is a rare disease, with the ulcerative variant being most frequent. The lower legs are the most commonly affected sites. The recurrence rate in our study was about 46% regardless of the treatment prescribed. Pulmonary involvement was fatal in two patients.

Adolescent↗

Clinical manifestations of pyoderma gangrenosum associated with inflammatory bowel disease.

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition associated with inflammatory bowel disease. PG occurs rarely in IBD patients and there are insufficient data on the clinical manifestations of this disease with IBD. OBJECTIVE: To determine the incidence, clinical manifestations and treatment of PG in patients with IBD and the connection to IBD, its activity and extent. METHODS: All patients hospitalized with IBD at a university hospital during a 20 year period were evaluated for the occurrence of PG. RESULTS: Of 986 patients hospitalized for IBD 6 suffered from PG (0.6% incidence). Their average age was 37 with equal sex distribution and equal distribution of Crohn's disease and ulcerative colitis. PG appeared 6.5 years on average after diagnosis of IBD in all patients. The development of PG correlated with significant clinical exacerbation of IBD, the majority having active colitis at the onset of the PG. Extraintestinal manifestations of IBD occurred in half the patients (sacroillitis, peripheral arthritis and erythema nodosum). Pathergy was not elicited in any patients. Four patients had multiple skin lesions, frequently on the lower extremities. Diagnosis was made by skin biopsy in four patients. There was little correlation between amelioration of IBD and the skin lesions. Treatment consisted of high dose steroids and immunomodulatory drugs (cyclosporine, azathioprine and dapsone) in conjunction with topical treatment. CONCLUSIONS: PG is a rare extra-intestinal manifestation of IBD that coincides with the exacerbation of the intestinal disease but does not always respond to treatment of the bowel disease.

Adult↗

Treatment of pyoderma gangrenosum with infliximab in Crohn's disease.

Pyoderma gangrenosum (PG) is an ulcerating noninfectious disease of the skin seen in 1 to 5% of patients with inflammatory bowel disease. The pathogenesis of PG has yet to be determined but may be related to abnormal T cell responses and the production of TNF-alpha, a powerful proinflammatory cytokine. Infliximab, a chimeric monoclonal antibody to TNF-alpha, has been approved for the treatment of Crohn's disease. We present four patients with PG treated with Infliximab for fistulizing Crohn's in whom complete healing of PG was achieved. Four patients with active fistulizing Crohn's disease and PG were treated. All patients were females ranging in age from 48 to 60 years, with a mean age of 54 years. Three of four patients had PG lesions located on the lower extremities; one patient had peristomal disease. All patients had at least colonic involvement of their Crohn's. The patients received either a single infusion or a series of three 5 mg/kg Infliximab infusions. All four patients demonstrated rapid healing of PG within 4 weeks of the first infusion of Infliximab. PG healing followed improvement in bowel disease. Complete resolution without recurrence was noted in all patients. Rapid resolution of PG was noted in four female patients with fistulizing Crohn's disease treated with Infliximab. Healing was complete, without recurrence. The anti-TNF-alpha properties of Infliximab suggest that healing may be mediated by the drug's effect on cytokine pathways, perhaps by blunted T cell activation early in the inflammatory cascade. We suggest an independent effect of Infliximab on PG.

Adult↗

[Pyoderma gangrenosum over a saphenectomy scar in a cANCA-positive patient with diverticulitis].

Pyoderma gangrenosum is a chronic, painful, ulcerative skin disease of unknown etiology. In 50 % of the cases, it is associated with systemic disease, primarily with inflammatory bowel disease. It can develop over areas of trauma or surgical wounds, possibly because of local immunoreactant deposits. We describe the case of a cANCA-positive patient with colonic diverticulitis who developed pyoderma gangrenosum over a saphenectomy scar.

Aged↗

Pyoderma gangrenosum involving the head and neck.

In six patients with pyoderma gangrenosum, the head and neck region was a major site of ulcerative skin disease. In two patients, the disease was limited to this anatomic site. Corticosteroids were effective therapy in five cases. In one case, occurring in association with ulcerative colitis, total proctocolectomy was required to control ulcerative scalp disease. Detailed histologic examination of a primary lesion in one case with 0.5-micron sections demonstrated morphologic evidence of mast cell activation, suggesting that mast cells may contribute to the pathogenesis of the inflammatory process in pyoderma gangrenosum.

Adrenal Cortex Hormones↗

Bilateral pyoderma gangrenosum of the hand: treatment with dapsone.

The first reported case of bilateral pyoderma gangrenosum of the hands is presented. One lesion was treated with wound care only, with spontaneous healing after two months. The contralateral lesion which occurred one month later was treated with wound care and oral dapsone. Healing occurred between four and six weeks after the start of dapsone. It is suggested that this acts by limiting the necrotizing process, thus allowing earlier epithelialization. Pyoderma gangrenosum is rare, and treatment is non-surgical. It is important that it is recognized to avoid extension of the necrotizing process through ill-advised surgery.

Aged↗

Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum.

Twenty-five patients had superficial ulcerative and vegetative pyoderma with granulomatous histologic findings. Healing occurred without systemic corticosteroid therapy in all but three patients. All patients had clinical pyoderma gangrenosum. In five patients the lesion occurred after surgery of the skin. Histopathologic study of 40 biopsy specimens showed focal neutrophilic abscesses of the papillary dermis, often with peripheral palisading histiocytes and foreign-body giant cells. Pseudoepitheliomatous, vegetative hyperplasia and sinus tract formation were observed frequently. All patient had massive areas of plasmacytosis, and 13 had eosinophils. Granulation tissue, hemorrhage, and fibrosis were additional features in some areas. Foreign material in the lesions was considered unimportant, except in one patient with a starch granuloma. Therapy with local corticosteroids, minocycline, tetracycline, or sulfa drugs resulted in healing in 15 patients. We believe that we have identified a localized, limited form of chronic superficial pyoderma gangrenosum with verrucous and ulcerative lesions and a granulomatous histologic appearance that represents a unique pattern of this disease in some patients.

Abscess↗

[Pyoderma gangrenosum: treatment with plasma exchange (4 cases)].

Four patients with pyoderma gangrenosum were treated by plasma exchange. The series included one woman (cervical localization) and three men (sural localization in 1 case, multiple trunk and facial localizations in the second case and multiple, recurrent localizations on trunk and limbs in the third case). In 2 cases another disease was associated (ulcerative colitis in 1 case and Crohn's disease in the other). Between 6 and 12 plasma exchange sessions were carried out in combination with corticotherapy (1/2 mg/kg/day). Results in 2 cases were rated as very good: progression of the disease interrupted and no further pain after the 1st plasma exchange, rapid healing of lesions (a skin graft was necessary for a lesion of hand exposing tendons). A good result was obtained in the 3rd case with interruption of progression of lesions after the 1st plasma exchange but slower relief of pain. Treatment was considered a failure in the 4th case since there was no obvious regression in lesions or pain. Three of the 4 patients had no recurrences, one patient developing recurrences on 4 occasions responding well to plasma exchange each time. Pyoderma gangrenosum is a good indication for use of plasma exchange, which limits extension of ulcerating lesions, suppresses or reduces pain and decreases esthetic prejudice.

Adolescent↗

Pyoderma gangrenosum with carcinoid tumor.

A patient with the classic lesions of pyoderma gangrenosum was found on autopsy to have a carcinoid tumor of the ileum. The simultaneous occurrence of a carcinoid tumor with pyoderma gangrenosum is interesting because both conditions are associated with immunologic defects.

Aged↗

Wegener's granulomatosis presenting as pyoderma gangrenosum.

We report three cases of Wegener's granulomatosis presenting with cutaneous ulceration resembling pyoderma gangrenosum. Wegener's granulomatosis classically affects the upper and lower respiratory tracts and the kidneys. Skin involvement occurs in up to 50% of patients. Increased awareness that cutaneous involvement can take the form of pyoderma gangrenosum and that it can be a presenting sign may lead to more rapid diagnosis of Wegener's granulomatosis.

Adult↗

Pyoderma gangrenosum of the cervix.

BACKGROUND: Ulcerated cervical lesions are common findings in gynecology, but pyoderma gangrenosum presenting solely as a cervical ulcer is unusual. CASE: A 33-year-old nulligravida presented with postcoital bleeding and an extensive ulcerated lesion of the cervix that showed nonspecific inflammation on biopsy. After 6 months of topical steroid treatment, the lesion resolved completely. CONCLUSION: Pyoderma gangrenosum should be suspected in nonspecific cervical ulcers, especially those that worsen after surgical resection or ablation and then respond to steroid treatment.

Adult↗