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A defective uptake of oxygen by peripheral blood granulocytes during phagocytosis, indicating a subnormal phagocytic capacity, has been found in a patient with regional enteritis complicated by pyoderma gangrenosum (PG). During administration of clofazimine and granulocyte function normalized and the skin lesions healed. It is possible that a defective granulocyte function may sometimes be involved in the pathogenesis of PG and that a clofazimine-induced improvement in the function will favour healing of the lesions. The result of treatment in our patient and in other cases recently published indicates that the drug may be worth trying in PG.

Pyoderma gangrenosum is a relatively rare, destructive, inflammatory disease of unknown cause which may present as a purely cutaneous disorder or may be associated with an underlying internal disease (ulcerative colitis, Crohn's disease, rheumatoid arthritis, multiple myeloma, lymphoma and others). Four selected patients are described which reflect the clinical spectrum of this condition; these cases and a review of the literature serve as a background for analysis of pyoderma gangraenosum as an entity and a discussion of its pathogenesis.

A patient is described who developed multiple areas of inflammatory pyoderma on the face, leading to extensive ulceration. Repeated investigations failed to demonstrate any specific bacteria or fungus responsible and trials of treatment with various antibiotics proved unsuccessful. Histology revealed a granulomatous abscess-like lesion, and the condition resembled what has been described as malignant pyoderma. The patient was successfully treated with oral dapsone and intralesional steroids.

In this paper, two cases of chronic ulcerative colitis displaying systemic manifestations are discussed. Each case illustrates the refractoriness of the systemic manifestations to colectomy and the total resolution of the manifestations by additional proctectomy. The implications of these clinical observations in relationship to the aetiology and therapy of ulcerative colitis are discussed.

The exact mechanism involved in the pathogenesis of pyoderma gangrenosum (PG) still remains unclear, yet there is an increasing number of reports associating PG with immunologic abnormalities. A correlation between PG and myeloproliferative disorders has also been described. We describe a patient with chronic myelocytic leukemia in whom PG developed during the course of illness. We present an immunologic analysis of this case, speculation on the pathogenesis of PG, and a review of the literature. We report the futility of current therapeutic modalities in the treatment of PG.

In the approach to the pruritic patient, it is important to first determine if a pyoderma is present. A thorough investigation of the integument should be undertaken during the physical examination. There are a wide variety of clinical appearances of pyodermas, so the clinician should recognize the lesions that are suggestive of infection (epidermal collarettes, draining tracts, and so on). When infection is present, it is necessary to direct therapy at the pyoderma and then re-evaluate the patient. Any animal that presents with a pruritic pyoderma deserves proper therapy directed at the infection before the clinician can assess the role that the pyoderma is contributing to the pruritus. Rational therapy in these cases requires that one determine the cause of the itch rather than merely masking the symptom of pruritus.

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.

Pyoderma faciale is a distinctive entity. Twenty-nine patients with this process were seen in the Mayo Clinic from 1969 to 1980. Twenty-seven patients had follow-up that ranged from 1 month to 11 years, and twenty-two had follow-up of 3 years or more. Clinical features that characterize the patients were (1) female predominance, (2) onset later than teenage acne vulgaris, generally at 19 to 40 years of age, (3) rapid onset and progression, (4) facial involvement with sparing of the back and chest, (5) cysts, swelling, and purulent drainage with a lack of comedones, and (6) paucity of systemic complaints. Patients were treated with multiple forms of therapy simultaneously, often including Vleminckx packs, oral antibiotics, incision and drainage, ultraviolet B, and intralesional steroids. Of twenty-five patients available for follow-up at 1 year, twenty-three had achieved remission, though fifteen patients required ongoing treatment to maintain optimal control. Twenty-three patients had scarring as a sequela. Patients with pyoderma faciale represent a subset of patients with acne in whom the outlook is favorable with appropriate therapy.

We present a case report of a man who had an abscess-like lesion on his left foot. The lesion discharged pus, enlarged, and became thicker, so that we could not differentiate the real toes from the out-growths. Moraxella phenylpyruvica and Pseudomonas putrefaciens were identified; direct agglutination tests were postive M. phyenylpyruvica. He was given antibiotics and antituberculosis therapy and had topical treatments. The foul odor disappeared with treatment, and the lesions were removed surgically 3 months later. The M. phenylpyruvica finally disappeared after the surgery. The final condition of this patient was markedly improved, although he had some residual scar contracture.

Systemic disorders that have been associated with pyoderma gangrenosum include inflammatory bowel disease, rheumatoid arthritis, paraproteinemias, and hematologic malignancies. We report the case of a 55-year-old woman with pyoderma gangrenosum, IgA monoclonal gammopathy, and a cortisol-secreting adrenocortical carcinoma. Review of the literature revealed one previous case of pyoderma gangrenosum associated with a solid tumor; at autopsy, a carcinoid tumor and an adrenocortical adenoma were found. Our patient's rapid improvement after the carcinoma was resected and her subsequent disease-free course suggests that the two conditions were related. This case suggests that evaluation for underlying malignancy should be considered in patients with pyoderma gangrenosum.

Pustulotic arthro-osteitis is a rare syndrome manifested by palmo-plantar pustulosis and abnormalities involving the anterior chest wall, the spine, the sacroiliac and peripheral joints. We present the case of a patient with pustulotic arthro-osteitis who developed pyoderma gangrenosum. We believe this association has not been previously described in the literature.

A patient is reported who suffered from pyodermia fistulans sinifica (acne conglobata sinifica, acne tetrade) and who presented a severe congenital alpha-1-antitrypsin deficiency (phenotype ZZ). The possible significance of the protease inhibitor deficiency for the development of this disease is discussed. Alpha-1-antitrypsin deficiency has been observed in several dermatoses (panniculitis, cutaneous vasculitis, Ehlers-Danlos syndrome, acquired angioneurotic edema and cold urticaria). The insufficient neutralization of liberated leukocyte proteases has been considered to play an important role in these disorders. Besides the ultrastructural finding in the liver cell which is typical for alpha-1-antitrypsin deficiency crystals were found as are seen in the cholesterol ester storage disease. This was probably caused by an increased influx of fatty acids.

17 years ago, selective IgA deficiency was diagnosed in a 45-year-old woman suffering from dermatitis ulcerosa. In 1984, this patient developed oral cancer with metastases in the regional lymph nodes. Her long-term history of nicotine and alcohol abuse in combination with IgA deficiency seemed to be a risk factor for oral malignancy.

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.

A gammaAchi--paraproteinemia was found in a 73 year-old female patient with pyoderma gangraenosum. Since the paraporteins hitherto observed in pyoderma gangraenosum predominantly belong to the IgA class, a relationship may exist between pyoderma gangraenosum and this type of paraproteinemia.

Atypical forms of pyoderma gangrenosum (PG) and Sweet's syndrome (SS) (acute febrile neutrophilic dermatosis) have been separately reported in association with various forms of leukemia over the past decade. A case in which both atypical bullous PG and atypical SS occurred in a patient with myeloid metaplasia is presented, and the literature is reviewed concerning the association of these dermatoses with neoplasias of myeloid origin. The current case and review support the thesis that these dermatoses, when associated with myeloproliferative disorders, represent points on a continuum of noninfectious, nonmetastatic, inflammatory neutrophilic dermatoses that may occur in patients with derangements in myeloid cell proliferation.

The case is presented of a patient with busulfan (Myleran) treated myeloid leukaemia, who developed bullous pyoderma gangrenosum. Skin symptoms appeared at the time when treatment was discontinued due to signs of bone marrow depression. The pyoderma disappeared following treatment with systemic steroid.

Pyoderma gangrenosum developed in a 45-year-old caucasian woman who had an 18 year past history of seronegative polyarthritis without any visceral or haematologic associated abnormalities. A literature review revealed only 12 reported cases. Extensive laboratory evaluation failed to detect alterations of the immune functions. The presence of a vasculitis with C3 deposits suggested that levamisole may have played a role in eliciting the lesion. Clofazimine, administered for a period of one month, was a relative failure.

Malignant pyoderma of the right ear developed in a 58-year-old woman, and it spread insidiously over the entire right side of the face. Pyoderma gangrenosum was diagnosed on the basis of clinical and histologic findings and the patient's history of ulcerative colitis and sclerosing cholangitis. After successive therapeutic failures with a score of conventional approaches over an 18-month period, oral cyclosporine, 10 mg/kg/day, was started. Significant healing of the skin was noted within 1 month. There was an associated remission of the patient's cholangitis and inflammatory bowel disease. The dosage of cyclosporine was progressively tapered and then discontinued after 7 months, when healing was complete. No relapse occurred in the subsequent 14 months, and there was an associated remission of the patient's cholangitis and inflammatory bowel disease. Cyclosporine merits serious attention for treatment of both pyoderma gangrenosum and sclerosing cholangitis.

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.

Of the many acknowledged systemic complications of inflammatory bowel disease, arthritis, iritis and erythema nodosum are observed most commonly and considered parts of the natural history. Pyoderma gangrenosum is a more ominous, less common but similarly associated complication that classically occurs in the course of ulcerative colitis. Its rarity in Crohn's disease stimulated the following report.

The presence of subcorneal pustules in a solitary, indolent, crusted plaque, or in erythema annulare-like lesions with a trailing scale, is evidence of atypical psoriasis. When these lesions appear late in life there is a significant risk of generalized pustular psoriasis with a poor prognosis. A case is presented in whom both of these atypical forms occurred and who developed severe generalized disease. Clinical and histological features are described.

The authors report three personal cases of phagedenic pyoderma associated with hemopathy. Studying twenty-five other cases, described in the reviews, the question can be debated on three levels: 1) The clinical characteristics of hemopathic pyoderma remain non-specific. However blisters, pustules, and even vegetating lesions occur very often; 2) the etiology of hemopathy is subject to change; acute leukaemia or myeloproliferation syndrome. During the polyglobulars associated with pyoderma, anemia and myelofibrosis appear quite constantly; 3) finally on the pathogenic level, the recent works tend towards the hypothesis of a damage in the functioning of the polymorphonuclears hence displaying the increase in the inflammation during phagedenic pyoderma.

A 14-year-old boy with a 12-year history of episodes of sterile pyarthrosis and cutaneous inflammation and ulceration was found to possess a serum factor which enhanced the random migration of leukocytes in vitro. The serum factor was isolated by Sephadex G-200 gel filtration and found to have a molecular weight of approximately 160,000. This partially purified principle enhanced the random migration of purified normal human neutrophils or mononuclear leukocytes by up to 200% without influencing chemotaxis. Trauma or other stimuli may lead to an accumulation of this serum factor in some tissues of the patient with resultant excessive leukocyte influx and heightened local activity of the leukocytes in the inflammatory exudate.

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.