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PubMed1992

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↗
PubMed1985

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↗
PubMed1994

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↗
PubMed1991

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↗
PubMed1994

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↗
PubMed1996

Pyoderma gangrenosum in association with undifferentiated seronegative spondylarthropathy.

The cases of 2 women with pyoderma gangrenosum (PG) and undifferentiated seronegative spondylarthropathy (SpA) are described. These 2 cases, together with the recently reported case of PG and B27-positive psoriatic spondylarthropathy, suggest that PG may also occur in association with forms of seronegative SpA that are different from primary ankylosing spondylitis (AS) and AS associated with inflammatory bowel disease.

Adult↗
PubMed1999

Pyoderma gangrenosum successfully treated with cyclosporin A.

The subjects of the study are a 48-year old male and a 50-year female patient with the idiopathic form of pyoderma gangrenosum. Both patients were treated with Cyclosporin A (Sandimmun) as monotherapy. Complete resolution was achieved after three months' treatment. No serious side-effects were observed.

Cyclosporine↗
PubMed1996

Familial pyoderma gangrenosum presenting in infancy.

UNLABELLED: Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease that occurs in all age groups. Less than 0.4% of patients are infants and represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affected with PG all presenting during infancy. Unlike the older age group, the ulcers spared the legs but involved the buttocks, thighs and perianal area in all the infants. CONCLUSION: This is the first reported family with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to absence of underlying associated disorders and the tendency of the lesions to appear in areas where infants frequently have other dermatoses. PG characteristically involves the buttocks, thighs and perianal area and spares the legs.

Adolescent↗
PubMedDate not supplied

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↗
PubMed1986

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↗
PubMed1985

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↗
PubMed2000

[Pyoderma gangrenosum: and important differential diagnosis from wound infection. Case report of a life threatening course].

Pyoderma gangrenosum (PG) is a necrotizing and ulcerative skin disease of unknown cause. The pathogenesis is thought to be related to a defective immune response. The ulcerations appear spontaneously or after skin trauma. Development in a surgical wound can be misinterpreted as wound infection. However, in contrast to postoperative infections, treatment of PG requires immunosuppressive therapy. We report a case of PG following hip-joint surgery that was complicated by a severe life-threatening systemic inflammatory response syndrome (SIRS). Typically, wound and blood cultures were negative for pathogens. Prednisolone stopped the activity of the disease promptly.

Anti-Inflammatory Agents↗
PubMed1993

Pyoderma gangrenosum after coronary artery bypass grafting.

Pyoderma gangrenosum is a rare cutaneous disorder that may complicate recovery after a cardiac operation. The lesions appear to represent a necrotizing infection; however, they do not respond to surgical debridement and antibiotic therapy. The treatment is based on high-dose corticosteroids and concomitant treatment of any underlying systemic disease.

Aged↗
PubMed1989

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↗
PubMed1988

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↗
PubMed1995

Lymphocyte subset abnormalities in German shepherd dog pyoderma (GSP).

Peripheral blood lymphocyte subpopulations were studied in 12 German shepherd dogs suffering from deep pyoderma (GSP). Twelve other healthy but matched dogs were used as controls. GSP was found to be associated with an imbalance in the CD4 and CD8 subsets (respectively 37.3 +/- 8.7% and 28.6 +/- 6.6%, as compared to 47.5 +/- 8.8% and 19.3 +/- 4.0% in the controls). The activation markers were not affected by GSP. Moreover, analysis of the B-cell populations showed a striking decrease in the level of CD21 cells (5.5 +/- 3.3% of CD21+ lymphocytes, compared to 12.2 +/- 6.0 in the controls). This study suggests that the immunological imbalance observed in GSP may be associated with defective helper cells, and provides further evidence that dogs suffering from GSP are not immunologically normal reactors.

Animals↗
PubMed1992

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↗