Bullous pyoderma gangrenosum with chronic myelogenous leukemia: report of a case.
A case of bullous pyoderma gangrenosum is presented in which study of the patient led to the discovery of chronic myelogenous leukemia.
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A case of bullous pyoderma gangrenosum is presented in which study of the patient led to the discovery of chronic myelogenous leukemia.
A case of pyoderma gangrenosum of the perineum is presented. The patient's surgical treatment, hospital course, and 10-year follow-up are described. Unusual problems in grafting, perineal support, and patient emotional response are discussed.
Even though pyoderma gangrenosum (dermatitis ulcerosa) is still considered to be a polyetiological syndrome with uncertain pathogenesis, immunological processes are attributed to it. It serves as an indicator of an underlying internal disease. A more than incidental occurrence is found of inflammatory intestinal diseases such as colitis ulcerosa, inflammatory joint disorders, among hematological diseases predominantly myeloic leukemia, paraproteinemia with and without plasmocytoma as well as inflammatory vascular processes. A variety of other simultaneous diseases have to be considered as isolated case reports; it remains for future investigations to decide if these will be classified among the merely coincidental diseases or as various manifestations of a common immunological process.
A 47-year-old multiparous woman presented with endometriosis, true urinary incontinence, and the mucosal and skin lesions of pyoderma gangrenosum. The subsequent investigations, treatment and course are discussed, as well as the possible causal interrelationship between the above three conditions.
Eleven patients, 8 females and 3 males, aged 17-53 years with chronic recurrent pyoderma (mean duration of 8.4 years) unresponsive to a variety of therapeutic modalities, were treated with oral levamisole 1 . 5-2 . 5 mg/kg/day (100-200 mg daily) for 2 consecutive days every week. Five out of eleven patients (3 males and 2 females) demonstrated one or more host defense abnormalities including impaired polymorphonuclear (PMN) chemotaxis, impaired bactericidal activity against Staphylococcus aureus, decreased in vitro lymphocyte response to Phytohaemagglutinin (PHA) and low serum IgM and IgA. Seven of eleven patients showed clinical improvement following levamisole administration for 4-11 months. Two showed complete clearance of skin lesions while on levamisole and for a year thereafter; three showed marked clearance of lesions during levamisole therapy but recurred with mild disease 6 months after termination of levamisole therapy; and two showed improvement of lesions during therapy but recurred immediately after levamisole discontinuation. Levamisole treatment was also associated with complete in vitro correction of PMN bactericidal abnormality, improvement of PMN chemotactic abnormality and augmentation of in vitro lymphocyte response to PHA. Correlation between in vitro potentiation of host defense mechanisms and clinical response was noted. Significant probable side-effects necessitating discontinuation of therapy included transient elevation of liver enzymes in 2 patients and extensive hemorrhagic skin rash in one.
A nodular, centrally verrucous, noninfectious pyoderma of the dorsal aspects of the hands and fingers developed in a 66-year-old man with metastatic bladder carcinoma after palliative cisplatin therapy. The dermatosis was clinically similar to erythema elevatum diutinum but biopsy failed to show substantial leukocytoclastic vasculitis. The eruption responded to dapsone. The role of cisplatin in the induction of the dermatosis is speculative.
A case of much less frequent association of the pyoderma gangrenosum and granulomatous disease of the colon (Crohn's disease of the colon) is presented. A literature review is made about the association of the same with other gastrointestinal affections. It is brought up to date the possible atiopathogenical mechanisms and therapeutical effects.
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.
Hereditary polymorphic light eruption (HPLE) occurs in Indians of North and South America. Affected persons are sensitive to long ultraviolet radiation and therefore receive no substantial benefit from conventional sunscreens. We have treated 46 patients with HPLE at the Red Lake Reservation, Minn, with topically administered dihydroxyacetone and lawsone, orally given beta carotene, or both. Oral beta carotene afforded adequate photoprotection to 33 patients, and four additional patients were protected with the combined use of oral and topical agents. Epidemiologic studies support our proposals that HPLE is a causative factor in streptococcal pyoderma in the American Indian and may be associated with epidemics of streptococcal glomerulonephritis.
Pyoderma gangrenosum is a rare condition which is sometimes associated with ulcerative colitis and Crohn's disease. The pathological basis is not completely understood but it may be caused by vasculitis. The lesions are typically said to have purple overhanging edges and a necrotic base. The condition may also occur with rheumatoid arthritis, multiple myeloma and leukaemia. The treatment of choice is systemic steroids but it may also respond to azathioprine, minocycline or clofazimine.
Two patients had pyoderma gangrenosum and rheumatoid arthritis. Biopsy specimens of the cutaneous ulcers in the patients showed a necrotizing vasculitis. Complement (C3) and immunoglobulins were not detected in the skin lesions. There was no important impairment of cell-mediated immunity observed.
The authors present a case report of pyoderma gangrenosum. Antibiotics may be indicated, but are not curative. A review of the literature is also presented.
A case of pyoderma gangrenosum associated with idiopathic myelofibrosis is described. It is important to recognise it, since the treatment with corticosteroids and sulphonamides is frequently effective.
Perianal pyoderma (hidradenitis suppurativa-acne conglobata) is a chronic disease which in its progressive form cannot be treated by conservative methods. Extensive suppurative foci must be radically excised incl. all systems of fistulas right down to the fascia and the defects which develop are allowed to heal per secundam or after several weeks they can at a different period of time be covered by a skin transplant. The authors present their 30-year experience with surgical treatment of 39 patients. Treatment was in all instances successful after radical surgical excision. All these patients have fully recovered as regards work capacity as well as social aspects.
The pathophysiology, clinical features, and therapy of the common pyodermas, those caused by Staphylococcus aureus and Streptococcus pyogenes, are reviewed.
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.
The effect of trimethoprim/sulfamethoxazole (T/SMX, 30 mg/kg, PO, q 12 h for 6 weeks) on thyroid function was evaluated in 21 dogs with pyoderma and normal baseline serum thyroxine concentrations. The population mean serum thyroxine concentration, but not the population mean serum triiodothyronine concentration, was significantly decreased at the end of treatment. After 6 weeks of treatment, the response in 3 dogs to thyrotropin administration was substantially reduced. Radionuclide thyroid imaging of 2 dogs after T/SMX treatment revealed higher-than-normal thyroid technetium 99m pertechnetate uptake, suggestive of an interference with iodide metabolism. Use of T/SMX may cause hypothyroidism, and inadequate thyroid function may be incorrectly diagnosed in dogs treated with this antimicrobial.
Pyoderma gangrenosum in a twenty-nine-year-old woman with associated long-standing Crohn's disease was treated with topical 5-aminosalicylic acid cream. Significant improvement in the ulceration occurred despite progressive deterioration of the inflammatory bowel disease. The possible mode of action is reviewed. The efficacy, together with the lack of side effects, warrants further study into the therapeutic role of topical 5-aminosalicylic acid.