1/227. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature. Pyodermatitis-pyostomatitis vegetans is a benign, rare disorder characterized by a pustular eruption in the oral mucosa and vegetating plaques involving the groin and axillary folds. Its association with inflammatory bowel disease is well established. We report the case of a 49-year-old-white man with ulcerative colitis who manifested a vegetating, annular plaque in the left inguinal region of 2 months' duration. Oral examination disclosed an erythematous mucosa with multiple painful pustules involving the labial and gingival mucosa. Histopathologic study demonstrated epidermal hyperplasia and an inflammatory infiltrate composed mostly of neutrophils and eosinophils, grouped into microabscesses within the epidermis and with a bandlike configuration in the upper dermis. Results of direct and indirect immunofluorescence studies were negative. We discuss the differential diagnosis between pyodermatitis-pyostomatitis vegetans and pemphigus vegetans. ( info) |
| A patient with the classic lesions of pyoderma gangrenosum was found on autopsy to have a carcinoid tumor of the ileum. The simultaneous occurrence of a carcinoid tumor with pyoderma gangrenosum is interesting because both conditions are associated with immunologic defects. ( info) |
3/227. Treatment of pyoderma faciale with isotretinoin in a patient with ulcerative colitis. The explosive onset of fluctuant facial papulonodules, usually in young women, is characteristic of pyoderma faciale. This disorder is neither a true pyoderma nor a variant of acne, but rather a severe form of rosacea. The most effective therapeutic modality appears to be isotretinoin, especially if preceded by a brief course of oral corticosteroids or a short interval of application of potent topical corticosteroids. Despite our concern about the potential adverse effects of systemic retinoids on underlying inflammatory bowel disease, isotretinoin was given to a patient with refractory pyoderma faciale. Response was dramatic, and no ill effects were encountered. ( info) |
4/227. Lupus erythematosus vegetans. A 29-year-old woman with known systemic lupus erythematosus presented with exudative and vegetative plaques bilaterally on her groins. The clinical and histological findings indicated a diagnosis of pyodermatitis vegetans. Direct immunofluorescence studies revealed the presence of a definite basement membrane zone band for IgG, IgM and C3, favouring lupus erythematosus. We propose the term lupus erythematosus vegetans for this combination of clinicopathological and immunohistological findings. ( info) |
5/227. rosacea fulminans in a patient with Crohn's disease: a case report and review of the literature. rosacea fulminans is a rare disorder of unknown cause that mainly affects postadolescent women, with abrupt onset and disfiguring course if left untreated. The simultaneous occurrence of rosacea fulminans and inflammatory bowel disease is rare and has been reported predominantly in the setting of ulcerative colitis. We describe here a case of rosacea fulminans in a patient with Crohn's disease and discuss a possible association between the two conditions. ( info) |
6/227. Squamous cell carcinoma arising in chronic perianal pyoderma a case report and review of Japanese literature. We report a rare case of squamous cell carcinoma developing from fistules of chronic perianal pyoderma in a 49-year-old Japanese man. He first noticed an abscess and nodule on his buttocks and perianal area 21 year previously (at the age of 28); the fistules formed later. These fistules were surgically removed, and an artificial anus was constructed 14 years ago (at the age of 35) in our hospital, when a histopathological examination revealed no malignant changes. However, he was recently admitted to our hospital with arterial bleeding from the ulcer of the buttock. On admission, the histological diagnosis of the ulcer was well differentiated squamous cell carcinoma. Wide local excision of the ulcer and scar tissue, including the sacrum, was performed. The defect was covered with a left latissimus dorsi flap and skin graft. He received radiation therapy after the operation. However, he died of cachexia and pneumonia. This case indicated that the CPP would better have been treated with wide excision before the development of SCC. Therefore, we recommend careful follow-up of patients affected by CPP and repeated biopsies of the lesion, particularly when the condition is severe, longstanding, and extensive. We discussed the term "CPP" and reviewed 22 cases of SCC arising in CPP reported in the Japanese literature. ( info) |
7/227. Extraintestinal Crohn's disease: case report and review of the literature. Crohn's disease is a granulomatous inflammatory bowel disease. Its pathologic findings include noncontiguous chronic inflammation and noncaseating granulomas. Any segment of the gastrointestinal tract can be involved, but it is uncommon to find that Crohn's disease has spread beyond the intestine. We describe the case of a man with extraintestinal Crohn's disease that was marked by quiescent involvement of the lower gastrointestinal tract and florid involvement of the nasal cavity, supraglottic structures, glottis, and skin. ( info) |
8/227. Pyoderma vegetans and ulcerative colitis. Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget's disease. Pustular eruptions associated with ulcerative colitis are reviewed. ( info) |
9/227. Pyodermatitis-pyostomatitis vegetans: a clinical course of two decades with response to cyclosporine and low-dose prednisolone. Pyodermatitis-pyostomatitis vegetans is a rare, polymorphous inflammatory disorder of the skin and oral mucosa first described by Hallopeau in 1898. On the skin papules, pustules and reddish brown annular vegetating plaques develop, most frequently in the intertriginous areas. In the mouth, yellowish flat ulcerations arise, typically in the shape of "snail tracks". The association with inflammatory bowel disease is very common. An unusual case with a chronic relapsing course of 2 decades is presented. Gastrointestinal inflammation was absent. prednisolone in high and medium doses suppressed most lesions. Various attempts with other drugs (dapsone, isotretinoin, azathioprine) to reduce the corticosteroid dose failed. This is the first report of the successful treatment of pyodermatitis-pyostomatitis vegetans with cyclosporin A, which proved to be highly effective in this regard. The unknown aetiopathology of pyodermatitis-pyostomatitis vegetans is discussed. ( info) |
| 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. ( info) |