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[CD8-positive, CD30-negative cutaneous T-cell lymphoma simulating pyoderma gangrenosum].

Ulcerated primary cutaneous lymphomas are not rare, but the clinical manifestation as a pyoderma gangrenosum look-alike is extraordinary. CD8-positive lymphomas are rare, unclassifiable tumours with variable prognosis. We report on a 49-year-old patient with a large ulcerated primary cutaneous lymphoma on the left chest wall presenting as pyoderma gangrenosum. With immunohistochemical staining, most lymphocytes were shown to be CD8-positive. The CD30 antigen was not expressed. After radiotherapy with complete skin irradiation, the lymphoma regressed completely. The patient has been free of relapse for 28 months so far.

CD8 Antigens↗

Direct immunofluorescence in pyoderma gangrenosum.

Direct immunofluorescence was done in fifty-one cases of pyoderma gangrenosum. Biopsy specimens were taken from the peripheral erythematous zone of the lesion. In thirty-one cases (61%), there was positive immunofluorescence, with perivascular deposition of immune reactants being the most frequent pattern (twenty-seven cases). These findings support a vasculitic pathogenesis of pyoderma gangrenosum.

Fluorescent Antibody Technique↗

Pyoderma gangrenosum presenting as Fournier's gangrene.

We report a case of pyoderma gangrenosum presenting as Fournier's gangrene. Although both processes have a similar presentation effective management is markedly different. Whereas broad-spectrum antibiotics and aggressive surgical débridement are necessary to control Fournier's gangrene, immediate institution of corticosteroids and local wound care are indicated for pyoderma gangrenosum.

Adult↗

Pyoderma gangrenosum complicated by herpes simplex virus infection.

High-dose immunosuppression used in the treatment of pyoderma gangrenosum predisposes patients to opportunistic infections. A 66-year-old man presented with recalcitrant pyoderma gangrenosum in which the ulcer itself became infected with herpes simplex virus type 1. This patient was immunosuppressed with multiple agents including topical and oral corticosteroids, cyclosporin, mycophenolate mofetil, intravenous immunoglobulin and infliximab. However, the patient's ulcer continued to extend despite this. It was not until the presence of this virus was detected using polymerase chain reaction on a viral swab of the lesion and oral aciclovir was commenced that the ulcer began to heal. In addition, a fungal granuloma developed on this patient's left forearm as a complication of the potent immunosuppression, which was resolved following treatment with oral voriconazole.

Acyclovir↗

Pyoderma gangrenosum associated with systemic lupus erythematosus: response to pulse steroid therapy.

Pyoderma gangrenosum in a 35-year-old woman with long-standing systemic lupus erythematosus was treated with pulse steroid therapy. Significant improvement in the ulceration and control of the lupus erythematosus occurred. The unusual association of these disorders is reviewed, and the efficacy of pulse therapy in the treatment of refractory cases of pyoderma gangrenosum is discussed.

Adult↗

Pyoderma gangrenosum: skin grafting after preparation with hyperbaric oxygen.

Four patients with pyoderma gangrenosum were treated with hyperbaric oxygen to prepare the wounds for skin grafting. Each wound responded to a course of daily hyperbaric oxygen with reduction of infection and increased capillary angiogenesis. During follow-up periods of 12 to 30 months, all wounds remained healed. Although the exact etiology of pyoderma gangrenosum is unknown, vasculitis with wound ischemia and infection are prominent components. Inspired oxygen partial pressures of 1100 to 1300 mmHg elevate wound oxygen tension despite relative ischemia. The impaired intracellular bacterial killing of hypoxic leukocytes is corrected during each day's 2-hour bolus of hyperbaric oxygen. Daily wound oxygenation increases collagen production by fibroblasts to support capillary angiogenesis.

Adolescent↗

Pyoderma gangrenosum associated with acne conglobata.

We report a 16-year-old male in whom pyoderma gangrenosum appeared in conjunction with acne conglobata. The patient also developed a seronegative spondyloarthropathy that was the main presenting complaint. There was no evidence of inflammatory bowel disease. Treatment with isotretinoin was successful. Both acne and pyoderma lesions healed and the articular symptoms improved. The present case, together with other reports in the literature show that acne conglobata must be included in the list of possible associations of pyoderma gangrenosum. We also comment on acne arthritis, a relatively frequent phenomenon, although still not generally known, in acne conglobata.

Acne Vulgaris↗

Sporotrichosis masquerading as pyoderma gangrenosum.

A 46-year-old man was diagnosed as having pyoderma gangrenosum after special stains and cultures from a skin biopsy specimen were reported as negative. Cutaneous sporotrichosis is usually diagnosed with relative ease on the basis of clinicopathologic features and prompt growth of the fungus in culture, although organisms are difficult to detect in tissue even with special stains. Identification of Sporothrix schenckii was delayed for three months in this patient because of unusual growth characteristics noted in the culture. The report of this patient's clinical course illustrates both the need to frequently reassess the diagnosis of pyoderma gangrenosum in treatment-resistant patients and the fact that S schenckii may be difficult to diagnose clinically and mycologically if the growth characteristics of the fungus are unusual.

Dermatomycoses↗

Prostatic infarction involving the urinary sphincter, an association with pyoderma gangrenosum.

A 70-year-old man presented with haematuria and restrictive urinary symptoms 7 years after a transurethral prostatectomy (TURP). Onset of symptoms coincided with development of a large skin ulcer on his back. A diagnosis of pyoderma gangrenosum was made based on clinical signs and histopathology. On repeated cystoscopy the urethral sphincter was obliterated by necrotic tissue, suspicious for malignancy. Histopathology revealed only necrotic tissue consistent with infarction of the prostate and sphincter. This clinical presentation with infarction of the prostate and sphincter, concurrent with development of skin ulcers, was consistent with pyoderma gangrenosum affecting the prostatic urothelium and sphincter. Previous prostatic surgery may have predisposed our patient to development of pyoderma gangrenosum in the area of the prostate.

Aged↗

Defective monocyte function in pyoderma gangrenosum with IgG kappa paraproteinaemia.

Peripheral blood monocytes from a patient with pyoderma gangrenosum and paraproteinaemia showed defective phagocytosis; longitudinal observations demonstrated an association between the defect, the level of paraprotein and the clinical activity of the pyoderma. Treatment with Melphalan led to a reduction in the paraprotein level and remission of the pyoderma and was accompanied by normalization of monocyte function. After 8 months remission the paraprotein level rose again and was accompanied by a recurrence of the monocyte defect; shortly after this the pyoderma recurred. Pre-incubation of normal monocytes with the patient's plasma or immunoglobulin fractions revealed that a similar defect could be induced in normal cells by the patient's monomeric IgG. The patient's serum also had anti-heparin activity and the relationship between this and the phagocytic defect was explored. These studies indicate a possible pathogenetic mechanism underlying the association between pyoderma gangrenosum and monoclonal gammopathy.

Aged↗

Interleukin-8 overexpression is present in pyoderma gangrenosum ulcers and leads to ulcer formation in human skin xenografts.

Interleukin-8 (IL-8) is a potent chemotactic polypeptide for neutrophils. However, the role of this cytokine during inflammation remains unclear. Skin specimens from patients with pyoderma gangrenosum demonstrated IL-8 overexpression in skin ulcers, which suggests a role for IL-8 in the development of the disease. We therefore constructed a recombinant adenovirus expressing the complementary deoxyribonucleic acid encoding human IL-8 (IL-8/Ad5) that induces a 2000-fold increase in IL-8 expression of infected human fibroblasts in vitro. Human skin engrafted to severe combined immunodeficiency mice and then injected with the recombinant virus demonstrated erythema, an intense perivascular infiltration of neutrophils, and extravasation of erythrocytes after 8 hours. By 12 hours after injection, neutrophils had accumulated beneath the epidermis, which then necrotized, and one or more ulcers that remained for approximately 2 weeks were observed. Clinically and histologically, the ulcers resembled pyoderma gangrenosum. These clinical and experimental findings suggest an etiologic role of IL-8 in the pathogenesis of pyoderma gangrenosum.

Animals↗

Minocycline hydrochloride hyperpigmentation complicating treatment of pyoderma gangrenosum.

Minocycline-associated hyperpigmentation is an uncommon side effect. We report the case of a patient with pyoderma gangrenosum successfully treated with oral minocycline but complicated by marked hyperpigmentation in his pyoderma gangrenosum and acne scars. One of the clinical forms of minocycline hyperpigmentation includes dark-blue or black macules in depressed acne scars or other sites of skin inflammation; this pattern seems to be independent of the total cumulative dose and the skin process.

Adult↗

Acute febrile neutrophilic dermatosis (Sweet's syndrome) and the related conditions of "bowel bypass" syndrome and bullous pyoderma gangrenosum.

Since Sweet's initial description of eight patients, the concept of acute febrile neutrophilic dermatosis has evolved and changed. We have expanded it to include patients with bullous pyoderma gangrenosum, bowel-bypass syndrome with or without the bypass, the vesiculopustular eruption or ulcerative colitis, and possibly even typical pyoderma gangrenosum. A variant of acute febrile neutrophilic dermatosis in which acute myeloid leukemia is present has been reported and seems identical to bullous pyoderma gangrenosum. Although no clear pathogenesis has been demonstrated, studies of the bowel-bypass syndrome have implicated immune complex disease. Therapy with prednisone is usually effective, but numerous other anti-inflammatory agents have been used effectively.

Acute Disease↗

Recurrence of pyoderma gangrenosum within a chronic wound following microvascular free-tissue transfer.

The authors present a 29-year-old woman with a chronic foot wound that failed to heal, despite extensive medical and surgical therapy. The diagnosis of pyoderma gangrenosum was ultimately made, and the patient was started on systemic cyclosporine therapy. In the absence of apparent active disease, surgical debridement and microvascular free flap reconstruction were performed to achieve wound closure. Six weeks postoperatively, recurrence of the pyoderma gangrenosum was identified in the free flap, resulting in partial, superficial, flap necrosis. Laboratory evaluation at that time demonstrated subtherapeutic cyclosporine levels. Once the cyclosporine level was increased to the therapeutic range, the wound healed, and the microvascular free flap was salvaged. Because of the relative lack of precision in both the clinical and pathologic determination of acuity level, as well as the tendency toward pathergy, surgical treatment of any form poses many potential risks for these patients. For this reason, surgery should serve only as an adjunct to medical therapy, which remains the mainstay for treatment of pyoderma gangrenosum.

Adult↗

Pyoderma gangrenosum associated with crohn disease: effect of TNF-alpha blockade with infliximab.

Eight patients with pyoderma gangrenosum associated with Crohn disease were treated with infliximab. All had active mucosal inflammation indicated by endoscopic examination. Within 1-4 months, infliximab treatment resulted in complete healing of the pyoderma gangrenosum in 3 cases (1 parastomal, 2 lower limb), partial healing in 3 (2 parastomal, 1 lower limb) and temporary improvement in 2. Adverse effects such as skin rash, pneumonia and diarrhoea were seen in three patients. Our results imply that infliximab has a therapeutic potential on skin manifestations associated with inflammatory bowel disease, even though successful treatment may require repeat courses of infliximab infusions.

Adolescent↗

Pyoderma gangrenosum.

The authors present a case report of pyoderma gangrenosum. Antibiotics may be indicated, but are not curative. A review of the literature is also presented.

Humans↗

Pyoderma gangrenosum of the head and neck.

Pyoderma gangrenosum is an ulcerative skin disorder usually associated with an underlying systemic disease. Head and neck involvement is rare, but possibly more common than once thought. The etiology of this disease is unclear, but may be related to an abnormal immunologic response. There are no pathognomonic histologic or laboratory findings; the diagnosis is made by the clinical appearance of the lesions and disease course. Treatment consists of immunosuppression and local wound care in addition to a search for and treatment of an underlying primary systemic disorder. A case report and review of the literature is presented with discussion of common head and neck manifestations, the differential diagnosis, and treatment alternatives.

Adult↗