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Does intestinal resection heal the pyoderma gangrenosum of inflammatory bowel disease?

A retrospective study of nine patients with active pyoderma gangrenosum at the time of operation for inflammatory bowel disease showed two patterns of postoperative skin healing: 1) prompt healing within 2 months, occurred in five patients with moderate to severe inflammatory bowel disease. 2) skin disease persisted in four others, healing only after a year. Three of these patients had mild ulcerative colitis, and in them, the operation was carried out in the hope of curing crippling pyoderma gangrenosum. The fourth patient had only an intestinal bypass for ileitis. Our observations suggest that prompt skin healing may occur after surgery in patients with severe inflammatory bowel disease, but not necessarily in those with milder bowel disease or in those in whom some bowel disease persists.

Adolescent

Pyoderma gangrenosum. Abnormal monocyte function corrected in vitro with hydrocortisone.

A 34-year-old man with chronic active hepatitis and pyoderma gangrenosum demonstrated abnormalities in neutrophil and monocyte function. Monocytes from this man had diminished chemotaxis and bacterial phagocytosis. These functions were significantly improved in vitro after one hour of incubation of monocytes with 10(-5) hydrocortisone. Neutrophil function was nornal for bactericidal activity and phagocytosis, but neutrophil chemotaxis was diminished. A plasma inhibitor was not found to explain these phagocyte alterations.

Adult

Pyoderma gangrenosum. Occurrence with altered cellular immunity and a circulating serum factor.

Aberrations of cellular immune functions in pyoderma gangrenosum (PG) may lead to nonspecific activation of inflammatory cells or to an imbalance of suppression leading to autoaggression (chronic ulceration). A patient with severe unremitting PG had anergy to a battery of seven skin test antigens. Mixed lymphocyte reactions, autologous mixed lymphocyte reactions, lymphocyte proliferative responses to antigens, and the production of leukocyte inhibitory factor were substantially suppressed, while the lymphocyte responses to mitogens were unaffected. Quantitative immunoglobulin and complement levels were normal. The inhibition of cellular immune functions was mediated by a factor in the patient's serum. This factor also inhibited lymphocyte functions of normal unrelated control subjects. Preliminary studies demonstrated that the factor is nondialyzable, heat stable, and not adsorbed by Staphylococcus A protein. Pulse therapy with large doses of corticosteroids resulted in dramatic clinical improvement.

Adult

Pyoderma faciale. A review and report of 20 additional cases: is it rosacea?

BACKGROUND AND DESIGN: Pyoderma faciale was originally described by O'Leary and Kierland in 1940. It is characterized by the sudden onset of monstrous coalescent nodules and confluent draining sinuses confined to the face of young women in their early 20s. This report summarizes our results in 20 cases. The women were 15 to 46 years old (mean, 25 years). RESULTS: All women were flushers and blushers. Histopathologic examination revealed a dense perivascular and periadnexial infiltrate, including granulocytes, eosinophils with epithelioid granulomas, and septal and lobular panniculitis. No consistent laboratory abnormalities were found. After much therapeutic experimentation, we developed an effective treatment plan, based on a combination of oral isotretinoin and corticosteroids. CONCLUSION: We regard it as an extreme form of rosacea and suggest it be renamed rosacea fulminans in analogy with its counterpart, acne fulminans.

Adolescent

Pyoderma gangrenosum involving the eyelid.

A 62-year-old man was seen with an ulcer of the left upper eyelid of two weeks' duration. Over the previous 40 years, similar ulcers had intermittently developed elsewhere on his skin and spontaneously resolved over several months leaving atrophic scars. A biopsy specimen of the eyelid lesion showed epidermal ulceration with acute inflammation and liquefactive necrosis of the underlying dermis. Microorganisms and vasculitis were not present. On the basis of the clinical history and a compatible biopsy specimen, the diagnosis of pyoderma gangrenosum was made. This is an uncommon, idiopathic, ulcerating skin disease that may also have ocular manifestations. Eyelid ulcers have not previously been reported, to our knowledge. The diagnosis is established by clinical history and exclusion of other causes of ulceration.

Eyelids

Peristomal pyoderma gangrenosum and inflammatory bowel disease.

Pyoderma gangrenosum (PG) is a debilitating skin disease most often associated with inflammatory bowel disease and is a reportedly rare cause of peristomal ulceration. The lesions of PG rapidly evolve from small, erythematous pustules to deep, painful, pyogenic ulcers within hours to days of onset. Although the behavior and the appearance of the lesions of peristomal PG are diagnostic, a lack of familiarity with PG often leads to misdiagnosis and inappropriate therapy. This study reports four cases of peristomal PG and discusses the 20 previously reported cases in patients with inflammatory bowel disease. Seventy-five percent of patients were female and 67% had Crohn's disease. All patients had colitis, including all of the patients with Crohn's disease, 82% of whom had additional perineal complications. The diagnosis of peristomal PG was based on clinical appearance alone in 83% of cases. The onset of peristomal PG ranged from 2 weeks to 3 years following ostomy. The response to medical therapy was variable. All cases (17 of 17) treated with high-dose corticosteroids and local wound care responded, but five cases required additional therapy. No patient was successfully treated with stoma revision. Risk factors for the development of peristomal PG include Crohn's colitis, female gender, and perineal disease. While most patients respond well to systemic steroids and local wound care, up to one third of patients require long-term medical management.

Adolescent

Bullous pyoderma gangrenosum after granulocyte colony-stimulating factor treatment.

The hematopoietic growth factors are under investigation for the treatment of patients with chemotherapy-induced bone marrow suppression. One such trial at the University of California, Los Angeles involves chemotherapy with or without granulocyte colony-stimulating factor (G-CSF) in patients with small cell lung cancer. The authors report a case of a patient who had bullous pyoderma gangrenosum at the site of previous eczema during treatment with G-CSF. The lesions resolved promptly when the drug was discontinued. Other investigators have recently reported inflammatory complications of G-CSF and granulocyte-macrophage colony-stimulating factor (GM-CSF) but this is the first case report of biopsy-proven neutrophilic dermatosis associated with administration of a hematopoietic growth factor. Patients should be monitored for development of inflammatory processes during G-CSF therapy and this therapy should be given with caution to those patients with existing inflammatory conditions.

Carcinoma, Small Cell

Pyoderma gangrenosum in association with psoriatic arthritis.

We present a patient with psoriasis and psoriatic arthritis who developed multiple large, painful ulcers that were scattered over his anterior lower legs and were characteristic of pyoderma gangrenosum (PG). This association has not previously been described. We review other forms of arthritis that usually occur with PG and discuss them in light of this observation.

Arthritis, Psoriatic

Pyoderma gangrenosum in Crohn's disease: report of a case.

A case of pyoderma gangrenosum of the abdominal wall occurring in a patient with histologically proven Crohn's disease of the colon is described. An aggressive surgical approach (wide excision with delayed skin grafting) resulted in rapid healing of the abdominal wall. Subsequent colectomy through the previously involved abdominal site was accomplished without complication.

Abdominal Muscles

The association of pyoderma gangrenosum with ulcerative colitis in Japan.

A patient with pyoderma gangrenosum (PG) and ulcerative colitis (UC) is described. He had melena and systemic skin lesions. The skin lesions consisted of small discrete ulcers on the back and head and large punched-out ulcers on the legs. He was successfully treated with prednisolone and salazosulfapyridine. He became asymptomatic after two weeks' treatment. Although the association of PG with UC is well documented among Caucasians, it is very rare among Japanese.

Adult

Thymopentin treatment in a patient with pluriorificial pyoderma vegetans.

This paper presents longitudinal clinical and immunological findings in a patient with a life-long history of pluriorificial pyoderma vegetans, a disease characterized by a distinct immunodeficiency of T lymphocytes. After treatment with thymopentin, 50 mg s.c. three times weekly for 12 weeks, the number of T lymphocytes in peripheral blood normalized, while other impaired immunological functions failed to improve, however. The most impressive observation was the significant clinical improvement of the patient's condition.

Adjuvants, Immunologic

Post-surgical pyoderma gangrenosum of the vaginal vault associated with ulcerative colitis and Behçet's disease; a case report.

A case is described in which a patient with ulcerative colitis developed chronic vaginal ulceration around the incision lines after cone biopsy. The ulcers persisted at the vaginal vault after abdominal hysterectomy and removal of a cuff of vagina. Non-specific histological features compatible with pyoderma gangrenosum were found on biopsy. The auto-immune nature of this chronic ulcer is further supported by its rapid response to steroid therapy and the subsequent development of Behçet's syndrome.

Adult

Immunologic aspects of German shepherd dog pyoderma (GSP).

In 21 dogs with clinical features of German Shepherd dog Pyoderma (GSP) parameters of the specific and aspecific immune system have been examined. Chemotaxis and killing capacities of neutrophilic leucocytes were undisturbed, whereas in skin biopsies no specific immunoglobulin or complement deposits were found with immunofluorescence. With double immunodiffusion, antibodies against Gram-positive bacteria were found. In a laser nephelometric assay significantly elevated levels of IgG, IgGab, IgGd, IgM and bacterial components, associated and non-associated with circulating immune complexes, were detected. However, no relation was found with the disease state. It is concluded that dogs with GSP are immunologically normal reactors. A bacterial hypersensitivity reaction is hypothesized as a possible initiating factor in the pathogenesis of GSP.

Animals

Fatal pyoderma gangrenosum in association with C7 deficiency.

Although pyoderma gangrenosum (PG) is often associated with systemic diseases, it has not been reported in association with congenital complement deficiencies. We describe an aggressive and ultimately fatal case of PG in a patient with a congenital C7 deficiency. Deficiencies of C7 can be associated with decreased neutrophil chemotaxis, phagocytosis, and opsonization, similar to the immunologic abnormalities described in patients with PG. Our patient's decreased complement level, if not directly related to the development of PG, may have contributed to the aggressive nature of her disease.

Adult

Epidemiology and control of staphylococcal pyoderma among newborn infants: evaluation of a method for routine cord care with 4 per cent chlorhexidine-detergent solution.

The incidence and types of staphylococcal purulent lesions were prospectively studied among 3602 newborn infants. During an epidemic of post-discharge pyoderma, 87 per cent of all Staphylococcus aureus isolates from lesions could be traced to the nursery. The risk of post-discharge disease was related to the degree of staphylococcal skin colonization in the nursery. A method for routine cord care in the nursery with 4 per cent chlorhexidine-detergent solution was evaluated and proved to be effective in reducing staphylococcal colonization in the nursery as well as purulent skin disease at home. The clinical effectiveness of the regimen was followed up for 19 months and a persistent low infection rate was obtained among infants in the post-discharge period.

Chlorhexidine

Sulfapyridine and sulfones decrease glycosaminoglycans viscosity in dermatitis herpetiformis, ulcerative colitis, and pyoderma gangrenosum.

Shortly after the introduction of sulfa drugs, sulfapyridine was found to have unique therapeutic properties, unrelated to antibacterial activity. Later, sulfones were found to share the same properties. The disorders initially improved were dermatitis herpetiformis, pyoderma gangrenosum, subcorneal pustular dermatosis, acrodermatitis continua, impetigo herpetiformis and ulcerative colitis. They were also sometimes helpful in many other disorders. They are effective in select disorders characterized by edema followed by granulocytic inflammation or edema followed by vesicle or bullae formation. The sulfones work in low doses in leprosy and their mode of action is not fully understood. Several pieces of experimental information are available. It is proposed that these drugs are entering or influencing the protein moiety of glycosaminoglycans and decreasing tissue viscosity. This decreased tissue viscosity prevents edema and dilution of tissue fluid and decreases acute inflammation and vesicle and bullae formation.

Clofazimine

Pyoderma eczema and folliculitis with defective leucocyte and lymphocyte function: a new familial immunodeficiency disease responsive to a histamine-1 antagonist.

A new familial immunodeficiency disease characterised by recurrent and persistent pyoderma, folliculitis, and atopic dermatitis is described in a father and son. It is accompanied by abnormalities of lymphocyte function (including defective proliferative responses to phytomitogens, and subnormal response in immunoglobulin production after stimulation of the lymphocytes by pokeweed mitogen) and defective leucocyte chemiluminescence responses, which were associated with defective ability for intracellular killing of microbial organisms. The abnormalities of lymphocyte and leucocyte function, as well as the clinical manifestations, responded dramatically to treatment with the histamine-1 antagonist, chlorpheniramine, suggesting that the underlying defect in this disease may relate to defective histamine metabolism or abnormal expression of histamine receptors on lymphocytes and leucocytes.

Blepharitis

Pyoderma gangrenosum treated with clofazimine. Report of three cases.

Three cases of pyoderma gangrenosum (PG) responsive to clofazimine are reported. The ulcers were recalcitrant to other forms of therapy, and the patients were incapacitated. Healing began within 2 or 3 weeks. To our knowledge, these are the first reported cases of PG responsive to clofazimine in the United States.

Chronic Disease