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Pyoderma gangrenosum with large circumferential perianal skin loss in a child.

Pyoderma gangrenosum is an uncommon skin disorder characterised by deep ulcers surrounded by a violaceous over-hanging edge. Although in many instances there is no clear association with any underlying disease, pyoderma gangrenosum has been described in ulcerative colitis, Crohn's disease, polyarthritis, diabetes mellitus and myeloma. Pyoderma gangrenosum may also be seen as a rare manifestation of myeloproliferative disease including leukaemia. In children, as in our case, it may be the presenting feature.

Anal Canal↗

[Pyoderma gangrenosum--positive effect of cyclosporin A therapy ].

Although pyoderma gangrenosum is a disorder known since over 70 years, it still remains a diagnostic and therapeutic problem. We describe three subjects with pyoderma gangrenosum; two were females, one was male, one case was associated with colitis ulcerosa, two were without any related disorders. Histopathologic examinations supported the diagnosis in all cases. In spite of intensive topical and systemic treatment with corticosteroids, Dapsone, Clofazimine, no sufficient effects were achieved. Cyclosporin A introduced in the dose of 5 mg/kg/d resulted in dramatic response and complete remission. Serum CyA levels, biochemical parameters of liver and kidney function, blood pressure were monitored during the therapy. No adverse events due to Cyclosporin A were observed.

Administration, Topical↗

Pyoderma gangrenosum and monoclonal gammopathy.

The records of eight patients with pyoderma gangrenosum and monoclonal gammopathy showed that all patients except one had an IgA paraproteinemia. To date, seven patients have had a benign course and multiple myeloma has developed in one. In seven patients, the onset of the pyoderma gangrenosum preceded the detection of the monoclonal gammopathy. The monoclonal gammopathy did not seem to influence the morphologic findings, course, or therapy of the pyoderma gangrenosum. In the one patient with myeloma, treatment of the myeloma caused accelerated healing of the skin lesions.

Adult↗

Pyoderma gangrenosum associated with ulcerative colitis: response to infliximab.

Pyoderma gangrenosum is an extraintestinal manifestation of inflammatory bowel disease that can be therapeutically troublesome. We comment on the case of a patient with clinically inactive ulcerative colitis who progressively developed necrotic lesions on both tibial aspects of his legs, which corresponded both clinically and histologically to pyoderma gangrenosum. Treatment with steroids and azathioprine could not control this complication. A single dose of infliximab 5 mg/kg was given, achieving an impressive response of the skin lesions followed by complete healing 3 months later. Infliximab can be useful in the management of refractory extraintestinal manifestations of inflammatory bowel disease.

Antibodies, Monoclonal↗

[Clinical analysis of 6 cases of pyoderma gangrenosum].

This article reports 6 cases of pyoderma gangrenosum of either acute or chronic clinical type. Etiologically, we found that minor local trauma could induce ulcerative and destructive lesions, with typical pyoderma gangrenosum features following in 4 cases. All the patients responded well to systemic corticosteroids. In comparison with cases reported in other countries, no systemic diseases (including ulcerative colitis, Crohn's disease and polyarthritis) were found in our series. Cultures taken from early pustular lesions were sterile. Histopathological examination showed heavy neutrophilic infiltration in the dermis, and no evidence of vasculitis was found in the biopsies. Immunological investigations revealed no specific reactions in our cases.

Adolescent↗

Multiple pulmonary nodules in association with pyoderma gangrenosum.

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.

Anti-Inflammatory Agents↗

Annular crystalline keratopathy in association with immunoglobulin therapy for pyoderma gangrenosum.

PURPOSE: To report on a patient with a clinical presentation of annular crystalline keratopathy after immunoglobulin therapy for pyoderma gangrenosum. METHODS: Case report of a 6-year-old boy with biopsy-proven history of pyoderma gangrenosum who had undergone several cycles of systemic immunoglobulin therapy. The literature on ocular manifestations associated with pyoderma gangrenosum was reviewed. RESULTS: One year after the last cycle, the patient complained of the sudden onset of photophobia. Slit-lamp biomicroscopy revealed bilateral symmetric crystalline deposits in an annular region of the cornea. Because visual acuity was not reduced, specific therapy was not initiated. Symptoms could be reduced by the prescription of medical edged filter lenses. CONCLUSIONS: Annular crystalline corneal deposits may be associated with immunoglobulin therapy or represent a hitherto unknown ocular complication of pyoderma gangrenosum.

Child↗

Accelerated healing of pyoderma gangrenosum treated with bioengineered skin and concomitant immunosuppression.

Pyoderma gangrenosum is a rare, destructive, neutrophilic dermatosis, the origin of which remains largely obscure. The ulcerative variant of this inflammatory disorder causes painful, necrotic, rapidly enlarging ulcers. Because of pathergy, many clinicians avoid managing these nonhealing ulcers with aggressive surgical debridement and autologous grafts. This article proposes that the application of an allogeneic cultured human skin equivalent (Graftskin) not only circumvents this problem, but also hastens re-epithelialization of the ulcer bed. An added benefit of the possible improvement of the cosmetic appearance of the final scar by preventing severe wound contracture is also postulated. We report a newly diagnosed case of ulcerative pyoderma gangrenosum; the use of bioengineered skin as an adjunct to concurrent immunosuppressive therapy with cyclosporine hastened the healing and diminished pain in a rapidly enlarging leg ulcer. Within 2 weeks, the ulcer was 30% to 40% healed, achieving 100% re-epithelialization within 6 weeks.

Adult↗

[Pyoderma gangrenosum].

GENERAL CHARACTERISTICS: Rare cause of cutaneous ulceration, pyoderma gangrenosum is among the group of neutrophilic dermatites characterized by the richness of the mature neutrophilic polynuclear dermal infiltrate. The primary lesion is a pustule sitting on an inflammatory base; extremely painful, it rapidly ulcerates. The lesion rapidly extends to more than 10 cm in diameter, has a regular, sharp border and a peripheral roll of flesh exhibiting purulent channels on the inside and a red granulous surface often covered with a pustular coating. Little is known of this disease. CONCOMITANT AFFECTIONS: In more than 50% of cases, pyoderma gangrenosum is associated with other diseases, which must be systematically searched for. These may be digestive, essentially inflammatory enterocolitis with frequent development of peristomal ulceration, rheumatismal affections notably rheumatoid arthritis, hematological affections (benign monoclonal gammapathy, chronic myeloid hemopathy). FROM A PARACLINICAL POINT OF VIEW: There are no specific examinations. A cutaneous biopsy should be performed in all cases, notably to eliminate other causes of ulceration. Since concomitant disease can be subsequently revealed, it is essential to renew the paraclinical investigations, even after the disease has healed. NO CODIFIED TREATMENT: Treatment of the cause, if it can be cured, may be sufficient to permit regression of the lesions. Local treatments to provoke budding and hence avoid surinfection are mandatory. In the progressive and extensive forms, systemic treatment, notably high dose corticosteroids, is indicated. Surgery, a priori, is excluded.

Biopsy↗

Malignant pyoderma or pyoderma gangrenosum of the head and neck?

Malignant pyoderma is a rapidly progressive ulcerating process of unknown origin that predominantly affects the head and neck of young adults. Malignant pyoderma has been considered distinct from pyoderma gangrenosum because of the predominant head and neck location of the ulcers and because the ulcers lack undermining and surrounding erythema. A case of a 22-year-old woman with a severe ulcerative process predominantly affecting the head and neck is described. A review of the literature indicates that malignant pyoderma and pyoderma gangrenosum are almost certainly identical disorders.

Adult↗

Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder: report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy.

The term peripheral ulcerative keratitis represents a spectrum of inflammatory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only been reported in patients with pyoderma gangrenosum. Separate episodes of pyoderma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over the past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. Cyclosporine (4 mg/kg/d) treatment was started on confirmation of pyoderma gangrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the administration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pustular vasculitis with peripheral ulcerative keratitis. This rare ocular manifestation and the serious sequelae when left untreated make recognition crucial. Cyclosporine proved to be a very effective treatment for all of our patient's diseases.

Arthritis, Rheumatoid↗

Pyoderma gangrenosum.

Since its description 50 years ago, pyoderma gangrenosum has continued to capture the attention and imagination of all those who see its dramatic presentation. Clinical observation still provides the only reliable diagnosis. As investigative techniques increase, more and more intriguing immunologic abnormalities associated with this disorder are discovered, but understanding of the pathogenesis remains elusive. It is now recognized as an independent condition as well as a co-condition with many systemic disorders. Many new treatment options are available, allowing much individualization of treatment. For now, pyoderma gangrenosum remains an impressive, relatively easily recognized, but poorly understood disease.

Arthritis, Rheumatoid↗

Treatment of pyoderma gangrenosum with cyclosporin A.

Two patients with recalcitrant pyoderma gangrenosum were treated with oral cyclosporin A (5 mg/kg body-weight/day). Healing of the lesions was achieved in Patient 1 within 1 month of starting treatment, but new areas of ulceration appeared when the dose was reduced to 3 mg/kg body-weight/day. The ulcers showed marked improvement by 3 weeks after the start of treatment in Patient 2 and remained inactive at a maintenance dosage of 100 mg/day, but there was no change in the associated seronegative arthritis. A steroid-sparing effect of CyA was evident in both patients. It is suggested that a lower dose of cyclosporin A than doses used previously in the treatment of pyoderma gangrenosum may be equally effective.

Adult↗

Pustular pyoderma gangrenosum associated with ulcerative colitis in childhood. Report of two cases and review of the literature.

Pyoderma gangrenosum is a cutaneous disorder associated with systemic diseases such as ulcerative colitis, Crohn's disease, rheumatoid arthritis, and blood dyscrasias. We are reporting two cases of pustular pyoderma gangrenosum associated with ulcerative colitis. One patient had inactive bowel disease when she developed her third episode of pustules, erosions, and nodules on the left leg. The other patient exhibited a widespread painful vesiculopustular eruption that coincided with the onset of her colitis. Both patients presented with pustules as the primary manifestation of their pyoderma gangrenosum. Histologic examination of skin from both patients revealed an acute perifollicular inflammation. Pyoderma gangrenosum should be considered in the differential diagnosis of pustular disorders in children with underlying conditions such as ulcerative colitis.

Abscess↗

Pyoderma gangrenosum of the hand: a case series and review of the literature.

Pyoderma gangrenosum is a rapidly developing cutaneous ulcer that is rare in the hand and may mimic a fulminating infection. The correct treatment is nonsurgical; surgery may actually incite a pathergic response worsening the condition. Misdiagnosis results in inappropriate treatment, unnecessary surgery, and even amputations. The records of 7 patients with pyoderma gangrenosum of the hand treated at 2 large academic medical centers between 1992 and 1999 were reviewed. There were 4 men and 3 women with an average age of 58 years (range, 32-81 years). Five patients had bilateral involvement. Minor trauma preceded the appearance of the lesions. An associated systemic disease was found in all patients with ulcerative colitis being the most common (3 patients). All patients initially had a misdiagnosis of infection. Thirteen misdiagnoses (range, 1-3/patient) resulted in 16 unnecessary surgeries (mean, 2.2/patient) including 4 amputations and 2 failed skin grafts. No surgical procedure resulted in clinical improvement. All cultures were negative. Several physicians (mean, 5; range, 3-7) examined each patient before the final diagnosis of pyoderma gangrenosum, which was made by, or in conjunction with, a dermatologist in all cases. The average time to clinical improvement after correct medical treatment was initiated was 5 days.

Adult↗

[Pyoderma gangrenosum: a rare cause of unexpected wound healing delay].

Pyoderma gangrenosum represents a rare but severe disturbance of wound healing following operative interventions. A case report of pyoderma gangrenosum after breast surgery is presented and the aetiology and the various options regarding investigations and treatment are discussed considering the current literature.

Anti-Inflammatory Agents↗

Pyoderma gangrenosum in an elderly woman. Course marked by extensive morbidity.

A case of extensive medical and functional morbidity in an elderly patient during treatment of pyoderma gangrenosum is described. Pyoderma gangrenosum is an uncommon cause of ulcerative skin lesions often involving areas of skin trauma about the lower extremity. Because of the possibility of underlying disease and the special treatment requirements, the condition must be differentiated from other, more common causes of skin ulceration. Treatment includes local care, systemic corticosteroid therapy, and control of any underlying disease. Pyoderma gangrenosum serves as a model for the interactions of various types of morbidity in the healthcare of the elderly.

Administration, Oral↗